Cilium Assembly (Homo sapiens)

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RAB3IP:RAB11A:GTP:Golgi-derived vesicleCEP192 RAB8A:GDP:RAB3IP:RAB11A:GTP:FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsTTC30B DYNLRB1 FGFR1OP PLK1 PRKAR2B BBIP1 GTP HAUS7 TUBB4A MKS1 TCTEX1D2 HAUS5 YWHAE NDE1 C2CD3 NEK2 KIF3A HAUS3 DYNLL1 CEP152 RHO CEP135 CNTRL ARL3 DYNC2H1 CEP164 CEP72 DYNLRB1 TRAF3IP1 CEP72 HAUS7 DCTN1-2 TCTN1 TTC30A CLASP1 TTC8 HAUS3 IFT20 BBS1 KIF3A TUBG1 SFI1 CSNK1D TTC30B Golgi-derived vesicle CEP72 PLK1 RAB11A CEP164 HAUS5 CNGA4 TUBA4A IFT172 ODF2 NINL CEP83 TUBA4A MAPRE1 HAUS1 HAUS4 IFT20 BBIP1 CNGA2 TUBB4B DYNC1I2 PKD2 ARL3 PPP2R1A mothercentriole:C2CD3B9D2 MyrG2-CYS1 CEP76 ARF4 CEP192 DCTN3 IFT AHAUS8 CEP41 TCTEX1D2 DCTN2 DYNC1H1 TCTE3 CETN2 WDR60 SSNA1 ARL6 UNC119B GDP TCTE3 IFT46 UNC119BIFT80 TTBK2IQCB1 IFT57BBS4 IFT74NEK2 OFD1 MKS1 EXOC5 TCTN1 IFT27 TUBG1 TUBG1 CNGB1 YWHAG KIF3A CDK5RAP2 TMEM67 BBIP1 HAUS2 OFD1 IFT20 YWHAE CEP89IFT BBBS/CCT complexCEP72 RAB11A:GTP:Golgiderived vesicleGTP PRKAR2B RPGRIP1L EXOC2 MKKS TUBA1A GDP GDP HAUS3 BBSomeKinesin-2 motorsPPP2R1A RAB11A EXOC4 TUBA1A MAPRE1 acetylated microtubule ASAP1 FBF1 HAUS6 HAUS5 IFT52 NEK2 MicrotubuleB9D1 WDR35RABL5 IFT88 ARL13B:INPP5E:PDE6DCEP250 CLUAP CNGA4 EXOC2 NINL CCT3 CLASP1 acetylated microtubule CEP72 SCLT1 IFT122 CSNK1D MyrG2-NPHP3 PLK1 CNGA4 TTBK2 NEDD1 RAB8A RAB8A ARL13Bcentrosome:C2CD2:distal appendage proteins:TTBK2:MARK4DYNC1I2 AKAP9 DCTN3 HAUS4 CETN2 CEP192 SSNA1 FGFR1OP TUBA1A CKAP5 IFT88 WDR35 ASAP1 CCT5 CLUAPHAUS2 TTBK2 TNPO1 acetylated microtubule protofilament NDE1 DCTN2 TCTEX1D2 CEP192 WDR60 DYNLRB2 DYNC2H1 CEP76 CENPJ GTP TUBA1A BBIP1 PKD1 RAB11A RAB8A TTC30B NEDD1 CEP162 RAB3IP DYNLRB1 TRAF3IP1 FBF1 CSNK1D PCNT CCP110CEP250 TMEM67 retrograde IFTtrainsCoA-SHRAB8A:GTP:RAB3IP:RAB11A:GTP:RAB11FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsGTP DYNLRB1 NDE1 BBS2 CSNK1D GTP HSP90AA1 DYNLL1 CEP70 CEP70 GTP IFT52 TCTE3 IFT122SMO CEP78 CEP57 IFT80SDCCAG8 NEK2 GTP CSNK1E KIF3B PKD1 DYNC1H1 MyrG2-NPHP3 IFT43 HAUS2 TUBB RABL5TUBG1 IFT172 ASAP1 dimerUNC119B:myristoylated ciliary cargoMKS1 myristoylatedciliary proteinsDYNLL1 FGFR1OP PKD1 RHO CCP110 PCNT IFT140PCM1 RAB3IP:RAB8A:GDPIFT80 PKD1 Ac-CoAIFT88 IFT43 KIFAP3 HSPB11 AKAP9 CEP135 C2CD3 NDE1 ALMS1 UNC119B CEP250 CEP78 EXOC8 IFT74 AKAP9 ACTR1A KIF3C PPP2R1A CLASP1 IFT27HAUS4 DYNC1I2 ARL3 RAB3IP PKD2 PLK4 TUBA1A BBS1 IFT74 CEP152 CDK1 IFT88TUBA1A CNTRL DCTN2 ASAP1dimer:ARF4:GTP:VxPx-containing ciliary membrane proteinsHSPB11 CEP63 HAUS6 PCNT TCTEX1D1 NPHP1 CEP89 GTPCEP290 CEP83ALMS1 RAB3IPSMO NDE1 ALMS1 LZTFL1oligomer:BBSomeHAUS8 MyrG2-CYS1 PLK1 IFT27 TCTE3 KIFAP3 PRKAR2B IFT81 DYNC2LI1 RAB11A:GTP:RAB11FIP3dimer:ASAP1dimer:VxPx-containing ciliary membrane proteinsGTP CEP290 CLASP1 TUBA4A DCTN3 IFT52 PRKACA WDR34 TTC26 MARK4TCTEX1D1 FBF1IFT57 TUBG1 ARF4:GDPARF4:GTPBBS9 IFT172 TTC30B CNTRL KIFAP3 CKAP5 PPP2R1A IFT20 CEP192 TMEM216 CLUAP IFT B*DCTN1-2 HAUS4 DYNC2LI1 WDR35 BBS2 KIF3A DYNLRB1 TMEM216 IFT80 WDR60 CNGA2 DYNLL1 IFT140 BBS4 GTP TUBB4A NEK2 IFT140 CSNK1E PKD2 CDK5RAP2 IFT140 PLK1 CNGA2 TCTE3 CEP250 IFT46TUBB IFT57 CSNK1E RAB11A CLUAP BBS2 GTPALMS1 ASAP1 INPP5E DYNC1H1 TTC30A FGFR1OP WDR34 DYNLRB2 DYNC2H1 BBS2 BBS5 MARK4 CEP78 IFT43 PRKACA Centrosome:C2CD3:distal appendage proteins:TTBK2IFT27 ATATMCHR1 BBS5 HAUS8 TMEM216 IFT27 SFI1 WDR19 SFI1 TUBB BBS10 HAUS4 anterograde IFTtrainsDYNLL1 PDE6D:INPP5EPLK4 CEP63 CEP78 RAB3IP KIF3B KIF17 DYNC2LI1 TUBB4A WDR19 LZTFL1 oligomerTCTN2 CNGA4 DYNLL1 PKD2 CEP164 HAUS6 ODF2 CEP83 CEP78 GTP ACTR1A KIF17 Tectonic-likecomplexCNGA2 ARL6 CEP135 ODF2 DYNC1I2 YWHAE CNGA2 CNGA2 PPP2R1A CEP135 KIF3C TCTEX1D2 ALMS1 TRAF3IP1 TTC26 KIF17 dimerGolgi-derived vesicle RABL5 KIF3B PRKAR2B CEP97 RHO RHO AKAP9 CEP162 RAB11A CSNK1D RABL5 CENPJ CEP63 CLASP1 KIF17 CNGB1 DYNC1I2 WDR34 CDK5RAP2 TTC30B HAUS7 YWHAG BBS5 C2CD3 RABL5 RAB11FIP3 CEP72 IFT172 CEP89 GTP KIF17 IFT20 FGFR1OP HAUS7 BBS7CKAP5 CENPJ UNC119BTCP1 TRAF3IP1 PCNT TRIP11 TUBB4A DYNLRB2 KIF3B CEP164 DCTN1-2 ARL6:GTP:BBSome:ciliary cargoIFT27 TUBB4B NPHP4 EXOC7 AZI1 HAUS7 KIF17 CEP164 UNC119B:myristoylated ciliary cargoDYNC1I2 IFT57 CEP162 DCTN2 mother centrioleRHO IFT74 TCTEX1D1 exocystcomplex:RAB8A:GTP:RAB3IP:RAB11:GTP:RAB11FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsSSNA1 ARF4 CENPJ CLASP1 CNGB1 CCT4 PLK4 ARL13B GDPTUBA4A DCTN3 CSNK1E DYNC2LI1 PKD2 HSP90AA1 NEDD1 HAUS2 RAB8A RABL5 NINL DCTN1-2 basalbody:transitionzoneproteins:RAB3IP:RAB11A:GTP:Golgi-derived vesicleTTC26 DYNC1H1 BBS4 WDR35 KIF24 CEP76 ARL3 HAUS6 DCTN3 MCHR1 TTC26TTC30A AHI1 CNGA4 TUBG1 IFT52 CNGA4 RP2:ARL3:GDP:UNC119BLZTFL1 HAUS3 ALMS1 PLK4 CEP70 PCNT TCTN3 ALMS1 CENPJ CEP76 CEP70 TTC21B CEP97 HAUS3 CEP83 IFT57 CKAP5 HAUS5 NINL AZI1 SSNA1 SDCCAG8 KIF17 SSTR3 IFT88 INPP5E CEP250 KIF3A PPP2R1A MAPRE1 PAFAH1B1 PCNT CEP83 BBS7 CSNK1E ODF2 CSNK1E TRIP11MAPRE1 GDP TUBB NEK2 KIF17 YWHAG WDR34 KIF24 CEP63 DYNC1H1 IFT43 NPHP4 IFT BNINL TTC21B HAUS1 TUBB4B BBS5acetylated microtubule SSTR3 KIF17 dimer:TNPO1DYNC1H1 CCT8 TCTN1 AZI1 CNTRL RAB11FIP3 OFD1 CETN2 DCTN3 CEP70 EXOC1 BBS2 TCTEX1D1 HAUS7 DCTN1-2 FBF1 GTP CLASP1 TMEM67 acetylatedmicrotubuleDCTN2 BBS1 acetylatedmicrotubulePDE6DWDR34 TNPO1EXOC8 KIF3C BBS9 PKD1 ARL6:GTP:BBSome:ciliary cargoCCP110 TUBB4B NEDD1 DYNC2LI1 CEP162 CEP162 IFT20 PDE6D:INPP5EAKAP9 HAUS1 CNTRL IFT57 RAB8A:GDPKIF3C IQCB1 CEP192 CC2D2A OFD1 IFT81ASAP1 IFT57 TUBB CEP162 UNC119B PKD2 PAFAH1B1 NINL RAB11FIP3 TUBB4A KIF24 HSPB11 TTC30B IFT81 ARL3 AKAP9 PRKAR2B NEDD1 GTP CNGB1 SFI1 BBS9 TCTN3 DCTN1-2 YWHAG CKAP5 HAUS6 RAB3IP:BBSomeDCTN2 BBS12 DYNC2H1 ACTR1A RAB11A:GTPHAUS8 ARL3:GTP:UNC119B:myristoylated ciliary cargoHSP90AA1 HAUS4 TUBB ACTR1A DYNLL2 CDK1 TUBA4A MAPRE1 RP2:ARL3:GTP:UNC119B(active)exocyst complexCEP41 TRAF3IP1 Kinesin-2 motorsCNGB1 CSNK1D HAUS2 CNGA4 AHI1 CEP78 TCTN2 WDR19BBIP1DYNLL1 ARF4 RAB3IP TUBB4B CETN2 CNGA2 CNGB1 KIF3C PLK1 WDR35 IFT43 KIF3C CSNK1E ARL3 CNGA2 CNGA2 MAPRE1 PDE6D PKD1 AKAP9 EXOC5 TRAF3IP1 TTC21B UNC119B RHO CEP162 SFI1 CNTRL BBS9 CKAP5 IFT52 dynein-2retrograde IFTtrainsIFT46 HSPB11IFT ACEP70 SCLT1 WDR60 RPGRIP1L CDK1 SSNA1 DYNLL1 CSNK1D PPP2R1A RAB8A GBF1DYNC1H1 RAB11FIP3 IFT122 IFT172 CEP57 TUBB YWHAG AZI1 YWHAG CDK1 TUBB4B PRKAR2B DYNLL1 RAB11A:GTP:RAB11FIP3dimer:ASAP1dimer:ARF4:GTP:VxPx-containing ciliary membrane proteinsASAP1 MyrG2-CYS1 HAUS3 NEDD1 IFT20 SSNA1 NEDD1 TUBB CEP152 CEP97 PRKAR2B RAB11A OFD1 CCP110 PRKACA LZTFL1 MyrG2-NPHP3 TTC8 CEP250 DYNLRB2 DCTN3 PRKACA GTP TTBK2 Microtubule protofilament RAB11A KIF3A DYNLL2 CNGA4 HAUS6 DYNLRB1 CDK5RAP2 TUBB4A TCTEX1D1 ASAP1 ACTR1A CEP250 HAUS8 ASAP1 GTP EXOC3 CEP76 KIF17 PAFAH1B1 NPHP1 RAB8A:GTPHSPB11 C2CD3 PCM1 basalbody:transitionzone proteinsCEP290 HSPB11 AHI1 PKD1 KIF3B CEP57 SCLT1IQCB1 CEP152 DYNLL2 CNGA2 IFT80 CNTRL KIFAP3 TTC30B FGFR1OP CEP290 B9D1 DCTN1-2 HSP90AA1 CNGA4 WDR35 VxPx-containingciliary membraneproteinsKIF17 HAUS8 HAUS1 active kinesin-2motorsCEP135 TTC30B DYNC2LI1 HSP90AA1 MyrG2-NPHP3 BBS1TNPO1 PCM1 CEP72 ARL3:GTPGTP BBS7 ARF4 KIF3C IFT81 anterograde IFTtrainsDYNC2H1 EXOC1 IQCB1 MyrG2-NPHP3 TUBG1 CEP162 TUBG1 acetylated microtubule PKD2 YWHAE IFT122 IFT46 KIFAP3 MAPRE1 KIF24 TTC8 GDP WDR34 HAUS2 WDR19 PAFAH1B1 GTP ARL13B:INPP5EDYNC1I2 SFI1 SCLT1 IFT74 SCLT1 IFT80 TTC26 B9D2 BBS5 DYNC2H1 TCTE3 IFT43 Golgi-derived vesicle PCNT PPP2R1A HSP90AA1 KIFAP3 CEP41 CEP83 active dynein-2motorsCEP63 CEP41 DYNLRB1 TTC21B IFT74 CEP78 IFT140 RABL5 CEP135 PAFAH1B1 WDR34 PAFAH1B1 C2CD3 DYNC1I2 MyrG2-CYS1 IFT52 CEP164basal bodyHAUS2 IFT172 DYNC2H1 IQCB1 TTC8 BBS4 WDR19 RAB8A:GTP:RAB3IP:RAB11A:GTP:RAB11FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsCEP290 HAUS7 NDE1 FBF1 KIF24 ARL6 TUBB4A CEP97YWHAE IFT140 ODF2 GTPTCTN2 DYNLL2 IFT20IFT46 ACTR1A CEP57 NEDD1 dynein-2B9D1 TUBB4A YWHAE DYNC1H1 CEP41 DYNLL2 IFT172DYNLRB2 TUBA4A TTC21B DYNLL1 RAB11FIP3 ASAP1 HDAC6ALMS1 TUBB4B PKD1 PAFAH1B1 PCNT TTC30A HAUS3 DCTN3 acetylated microtubule IFT46 INPP5E CDK1 Centrosome:C2CD3:distal appendage proteinsCLUAP HSPB11 TTC26 CETN2 C2CD3 IFT81 ACTR1A CEP57 SFI1 IFT88 KIF24 KIF17 dimer:TNPO1BBS2RAB8A INPP5E HAUS8 DYNLL1 SDCCAG8 HAUS7 CEP57 IFT74 HAUS5 UNC119B CEP152 INPP5ECEP192 KIF3B CETN2 RP2 TUBB4B HAUS3 DYNLL1 CCP110 NPHP1 HAUS5 CEP152 YWHAG YWHAE PCM1 GDPWDR19 CEP41 CLUAP ARL13B CEP63 PCM1 HAUS4 IFT80 CENPJ ARL3 HSP90AA1 PDE6DCEP41 MARK4 CDK5RAP2 DYNLL2 AZI1 BBS9 CEP63 NPHP complexTRAF3IP1 CEP135 GDP CKAP5 myristoylatedciliary cargoBBS9RAB11FIP3 HSP90AA1 PRKACA CSNK1D MyrG2-CYS1 TTC30A IFT122 SEPT2DYNLL1 CEP290 SDCCAG8 SCLT1 CNGB1 RAB3IPPLK4 RHO KIF24 AZI1 CEP152 CENPJ B9D2 DYNC2LI1 GTP KIF17 CEP164 BBS7 CEP72 TCTEX1D2 TTC8 IFT122 FBF1 EXOC4 DYNLRB2 RAB11A NPHP4 FBF1 CEP63 SDCCAG8 CC2D2A GDPIFT52 TCTEX1D2 EXOC6 RP2:ARL3:GTP:UNC119BIQCB1 EXOC7 CEP135 ARF4 IFT81 TUBA4A CNGA4 BBS7 YWHAE IFT81 IQCB1 HAUS1 ODF2 IFT140 CEP41 CEP76 TTC26 TTC30HAUS8 BBIP1 CC2D2A TRAF3IP1RAB11FIP3 SDCCAG8 TCTE3 RPGRIP1L TTC30A PAFAH1B1 CDK5RAP2 CENPJ acetylated microtubule TUBA1A WDR35 HSPB11 PLK4 RAB3IP NEK2 UNC119B SSNA1 IFT122 IFT27 RAB3IP C2CD3 BBS4 CEP192 RAB11A CEP57 TCTN3 PLK4 CSNK1E RABL5 IQCB1 TTC30A VxPx-containingciliary membraneproteinsGTP IFT172 CNGB1 PCM1 SSNA1 CLUAP ACTR1A IFT52CDK1 FGFR1OP MAPRE1 BBS1 PKD2 TTC21BHAUS1 PRKACA WDR60 PDE6D CNGB1 DCTN1-2 CEP89 TUBA1A EXOC3 PKD1 ARL3:GDPCEP83 CCT2 AKAP9 KIF24 CEP89 C2CD3HAUS5 IFT74 MCHR1 RP2 HAUS6 CCP110 HAUS1 CEP250 ODF2 GTP TUBA4A CEP89 KIFAP3 OFD1 YWHAG BBSome ciliary cargoTCTEX1D1 CDK1 MARK4 HAUS6 CETN2 PKD2 ARL6:GTPOFD1 PDE6D RAB3IP PCM1 IFT46 CEP89 CEP76 IFT43CEP76 RP2 DCTN2 PCM1 CDK5RAP2 DYNLL2 CDK1 CEP152 RAB8A BBS7 SFI1 SDCCAG8 NDE1 CEP70 CEP97 TCTEX1D2 ARF4:GTP:VxPx-containing ciliary membrane proteinsIFT20:TRIP11PRKAR2B HAUS5 HAUS1 ARL3:GTP:UNC119BIFT80 HAUS2 MARK4 FGFR1OP CKAP5 TCTEX1D1 IFT88 DYNLL1 TTC21B CEP290 HAUS4 TTC30A GTP BBS4RHO DYNLRB2 PLK4 PLK1 NDE1 IFT27 IFT57 GTP RAB11FIP3 dimerBBS5 PKD1 WDR19 AZI1 SMO DYNLL1 RAB3IP IFT88 WDR60 H2ONINL CNGB1 CNTRL TTBK2 TTBK2 IFT81 TTC8SCLT1 RHO DCTN2 PRKACA NINL CLUAP OFD1 PiKIF3B SDCCAG8 UNC119B EXOC6 CEP97 PRKACA PKD2 AZI1 CEP57 RP2CEP70 PLK1 RAB11A CETN2 SSTR3 TTC26 BBS1 NEK2 CLASP1 1419, 28, 68, 98614745141141813513119, 28, 68, 98185, 14618147181818114


Description

Cilia are membrane covered organelles that extend from the surface of eukaryotic cells. Cilia may be motile, such as respiratory cilia) or non-motile (such as the primary cilium) and are distinguished by the structure of their microtubule-based axonemes. The axoneme consists of nine peripheral doublet microtubules, and in the case of many motile cilia, may also contain a pair of central single microtubules. These are referred to as 9+0 or 9+2 axonemes, respectively. Relative to their non-motile counterparts, motile cilia also contain additional structures that contribute to motion, including inner and outer dynein arms, radial spokes and nexin links. Four main types of cilia have been identified in humans: 9+2 motile (such as respiratory cilia), 9+0 motile (nodal cilia), 9+2 non-motile (kinocilium of hair cells) and 9+0 non-motile (primary cilium and photoreceptor cells) (reviewed in Fliegauf et al, 2007).

This pathway describes cilia formation, with an emphasis on the primary cilium. The primary cilium is a sensory organelle that is required for the transduction of numerous external signals such as growth factors, hormones and morphogens, and an intact primary cilium is needed for signaling pathways mediated by Hh, WNT, calcium, G-protein coupled receptors and receptor tyrosine kinases, among others (reviewed in Goetz and Anderson, 2010; Berbari et al, 2009; Nachury, 2014). Unlike the motile cilia, which are generally present in large numbers on epithelial cells and are responsible for sensory function as well as wave-like beating motions, the primary cilium is a non-motile sensory organelle that is present in a single copy at the apical surface of most quiescent cells (reviewed in Hsiao et al, 2012).

Cilium biogenesis involves the anchoring of the basal body, a centriole-derived organelle, near the plasma membrane and the subsequent polymerization of the microtubule-based axoneme and extension of the plasma membrane (reviewed in Ishikawa and Marshall, 2011; Reiter et al, 2012). Although the ciliary membrane is continuous with the plasma membrane, the protein and lipid content of the cilium and the ciliary membrane are distinct from those of the bulk cytoplasm and plasma membrane (reviewed in Emmer et al, 2010; Rohatgi and Snell, 2010). This specialized compartment is established and maintained during cilium biogenesis by the formation of a ciliary transition zone, a proteinaceous structure that, with the transition fibres, anchors the basal body to the plasma membrane and acts as a ciliary pore to limit free diffusion from the cytosol to the cilium (reviewed in Nachury et al, 2010; Reiter et al, 2012). Ciliary components are targeted from the secretory system to the ciliary base and subsequently transported to the ciliary tip, where extension of the axoneme occurs, by a motor-driven process called intraflagellar transport (IFT). Anterograde transport of cargo from the ciliary base to the tip of the cilium requires kinesin-2 type motors, while the dynein-2 motor is required for retrograde transport back to the ciliary base. In addition, both anterograde and retrograde transport depend on the IFT complex, a multiprotein assembly consisting of two subcomplexes, IFT A and IFT B. The primary cilium is a dynamic structure that undergoes continuous steady-state turnover of tubulin at the tip; as a consequence, the IFT machinery is required for cilium maintenance as well as biogenesis (reviewed in Bhogaraju et al, 2013; Hsiao et al, 2012; Li et al, 2012; Taschner et al, 2012; Sung and Leroux, 2013).

The importance of the cilium in signaling and cell biology is highlighted by the wide range of defects and disorders, collectively known as ciliopathies, that arise as the result of mutations in genes encoding components of the ciliary machinery (reviewed in Goetz and Anderson, 2010; Madhivanan and Aguilar, 2014).

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Reactome-Converter 
Pathway is converted from Reactome ID: 5617833
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Reactome version: 74
Reactome Author 
Reactome Author: Rothfels, Karen

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  152. Chiang AP, Nishimura D, Searby C, Elbedour K, Carmi R, Ferguson AL, Secrist J, Braun T, Casavant T, Stone EM, Sheffield VC.; ''Comparative genomic analysis identifies an ADP-ribosylation factor-like gene as the cause of Bardet-Biedl syndrome (BBS3).''; PubMed Europe PMC Scholia
  153. Humbert MC, Weihbrecht K, Searby CC, Li Y, Pope RM, Sheffield VC, Seo S.; ''ARL13B, PDE6D, and CEP164 form a functional network for INPP5E ciliary targeting.''; PubMed Europe PMC Scholia
  154. Jin H, White SR, Shida T, Schulz S, Aguiar M, Gygi SP, Bazan JF, Nachury MV.; ''The conserved Bardet-Biedl syndrome proteins assemble a coat that traffics membrane proteins to cilia.''; PubMed Europe PMC Scholia
  155. Blacque OE, Reardon MJ, Li C, McCarthy J, Mahjoub MR, Ansley SJ, Badano JL, Mah AK, Beales PL, Davidson WS, Johnsen RC, Audeh M, Plasterk RH, Baillie DL, Katsanis N, Quarmby LM, Wicks SR, Leroux MR.; ''Loss of C. elegans BBS-7 and BBS-8 protein function results in cilia defects and compromised intraflagellar transport.''; PubMed Europe PMC Scholia
  156. Ismail SA, Chen YX, Miertzschke M, Vetter IR, Koerner C, Wittinghofer A.; ''Structural basis for Arl3-specific release of myristoylated ciliary cargo from UNC119.''; PubMed Europe PMC Scholia
  157. Gruss OJ.; ''Nuclear transport receptor goes moonlighting.''; PubMed Europe PMC Scholia
  158. Snow JJ, Ou G, Gunnarson AL, Walker MR, Zhou HM, Brust-Mascher I, Scholey JM.; ''Two anterograde intraflagellar transport motors cooperate to build sensory cilia on C. elegans neurons.''; PubMed Europe PMC Scholia

History

View all...
CompareRevisionActionTimeUserComment
114640view16:10, 25 January 2021ReactomeTeamReactome version 75
113088view11:15, 2 November 2020ReactomeTeamReactome version 74
112322view15:24, 9 October 2020ReactomeTeamReactome version 73
101221view11:11, 1 November 2018ReactomeTeamreactome version 66
100759view20:37, 31 October 2018ReactomeTeamreactome version 65
100303view19:14, 31 October 2018ReactomeTeamreactome version 64
99850view15:58, 31 October 2018ReactomeTeamreactome version 63
99407view14:34, 31 October 2018ReactomeTeamreactome version 62 (2nd attempt)
99096view12:39, 31 October 2018ReactomeTeamreactome version 62
93627view11:29, 9 August 2017ReactomeTeamNew pathway

External references

DataNodes

View all...
NameTypeDatabase referenceComment
ACTR1A ProteinP61163 (Uniprot-TrEMBL)
AHI1 ProteinQ8N157 (Uniprot-TrEMBL)
AKAP9 ProteinQ99996 (Uniprot-TrEMBL)
ALMS1 ProteinQ8TCU4 (Uniprot-TrEMBL)
ARF4 ProteinP18085 (Uniprot-TrEMBL)
ARF4:GDPComplexR-HSA-5623424 (Reactome)
ARF4:GTP:VxPx-containing ciliary membrane proteinsComplexR-HSA-5620917 (Reactome)
ARF4:GTPComplexR-HSA-5623423 (Reactome)
ARL13B ProteinQ3SXY8 (Uniprot-TrEMBL)
ARL13B:INPP5E:PDE6DComplexR-HSA-5637977 (Reactome)
ARL13B:INPP5EComplexR-HSA-5624905 (Reactome)
ARL13BProteinQ3SXY8 (Uniprot-TrEMBL)
ARL3 ProteinP36405 (Uniprot-TrEMBL)
ARL3:GDPComplexR-HSA-5624072 (Reactome)
ARL3:GTP:UNC119B:myristoylated ciliary cargoComplexR-HSA-5624104 (Reactome)
ARL3:GTP:UNC119BComplexR-HSA-5624076 (Reactome)
ARL3:GTPComplexR-HSA-5624073 (Reactome)
ARL6 ProteinQ9H0F7 (Uniprot-TrEMBL)
ARL6:GTP:BBSome:ciliary cargoComplexR-HSA-5624112 (Reactome)
ARL6:GTP:BBSome:ciliary cargoComplexR-HSA-5624114 (Reactome)
ARL6:GTPComplexR-HSA-5624106 (Reactome)
ASAP1

dimer:

ARF4:GTP:VxPx-containing ciliary membrane proteins		ComplexR-HSA-5623429 (Reactome)
ASAP1 ProteinQ9ULH1 (Uniprot-TrEMBL)
ASAP1 dimerComplexR-HSA-5623427 (Reactome)
ATATProteinQ5SQI0 (Uniprot-TrEMBL)
AZI1 ProteinQ9UPN4 (Uniprot-TrEMBL)
Ac-CoAMetaboliteCHEBI:15351 (ChEBI)
B9D1 ProteinQ9UPM9 (Uniprot-TrEMBL)
B9D2 ProteinQ9BPU9 (Uniprot-TrEMBL)
BBIP1 ProteinA8MTZ0 (Uniprot-TrEMBL)
BBIP1ProteinA8MTZ0 (Uniprot-TrEMBL)
BBS/CCT complexComplexR-HSA-5624117 (Reactome)
BBS1 ProteinQ8NFJ9 (Uniprot-TrEMBL)
BBS10 ProteinQ8TAM1 (Uniprot-TrEMBL)
BBS12 ProteinQ6ZW61 (Uniprot-TrEMBL)
BBS1ProteinQ8NFJ9 (Uniprot-TrEMBL)
BBS2 ProteinQ9BXC9 (Uniprot-TrEMBL)
BBS2ProteinQ9BXC9 (Uniprot-TrEMBL)
BBS4 ProteinQ96RK4 (Uniprot-TrEMBL)
BBS4ProteinQ96RK4 (Uniprot-TrEMBL)
BBS5 ProteinQ8N3I7 (Uniprot-TrEMBL)
BBS5ProteinQ8N3I7 (Uniprot-TrEMBL)
BBS7 ProteinQ8IWZ6 (Uniprot-TrEMBL)
BBS7ProteinQ8IWZ6 (Uniprot-TrEMBL)
BBS9 ProteinQ3SYG4 (Uniprot-TrEMBL)
BBS9ProteinQ3SYG4 (Uniprot-TrEMBL)
BBSome ciliary cargoComplexR-HSA-5624113 (Reactome)
BBSomeComplexR-HSA-5617794 (Reactome)
C2CD3 ProteinQ4AC94 (Uniprot-TrEMBL)
C2CD3ProteinQ4AC94 (Uniprot-TrEMBL)
CC2D2A ProteinQ9P2K1 (Uniprot-TrEMBL)
CCP110 ProteinO43303 (Uniprot-TrEMBL)
CCP110ProteinO43303 (Uniprot-TrEMBL)
CCT2 ProteinP78371 (Uniprot-TrEMBL)
CCT3 ProteinP49368 (Uniprot-TrEMBL)
CCT4 ProteinP50991 (Uniprot-TrEMBL)
CCT5 ProteinP48643 (Uniprot-TrEMBL)
CCT8 ProteinP50990 (Uniprot-TrEMBL)
CDK1 ProteinP06493 (Uniprot-TrEMBL)
CDK5RAP2 ProteinQ96SN8 (Uniprot-TrEMBL)
CENPJ ProteinQ9HC77 (Uniprot-TrEMBL)
CEP135 ProteinQ66GS9 (Uniprot-TrEMBL)
CEP152 ProteinO94986 (Uniprot-TrEMBL)
CEP162 ProteinQ5TB80 (Uniprot-TrEMBL)
CEP164 ProteinQ9UPV0 (Uniprot-TrEMBL)
CEP164ProteinQ9UPV0 (Uniprot-TrEMBL)
CEP192 ProteinQ8TEP8 (Uniprot-TrEMBL)
CEP250 ProteinQ9BV73 (Uniprot-TrEMBL)
CEP290 ProteinO15078 (Uniprot-TrEMBL)
CEP41 ProteinQ9BYV8 (Uniprot-TrEMBL)
CEP57 ProteinQ86XR8 (Uniprot-TrEMBL)
CEP63 ProteinQ96MT8 (Uniprot-TrEMBL)
CEP70 ProteinQ8NHQ1 (Uniprot-TrEMBL)
CEP72 ProteinQ9P209 (Uniprot-TrEMBL)
CEP76 ProteinQ8TAP6 (Uniprot-TrEMBL)
CEP78 ProteinQ5JTW2 (Uniprot-TrEMBL)
CEP83 ProteinQ9Y592 (Uniprot-TrEMBL)
CEP83ProteinQ9Y592 (Uniprot-TrEMBL)
CEP89 ProteinQ96ST8 (Uniprot-TrEMBL)
CEP89ProteinQ96ST8 (Uniprot-TrEMBL)
CEP97 ProteinQ8IW35 (Uniprot-TrEMBL)
CEP97ProteinQ8IW35 (Uniprot-TrEMBL)
CETN2 ProteinP41208 (Uniprot-TrEMBL)
CKAP5 ProteinQ14008 (Uniprot-TrEMBL)
CLASP1 ProteinQ7Z460 (Uniprot-TrEMBL)
CLUAP ProteinQ96AJ1 (Uniprot-TrEMBL)
CLUAPProteinQ96AJ1 (Uniprot-TrEMBL)
CNGA2 ProteinQ16280 (Uniprot-TrEMBL)
CNGA4 ProteinQ8IV77 (Uniprot-TrEMBL)
CNGB1 ProteinQ14028 (Uniprot-TrEMBL)
CNTRL ProteinQ7Z7A1 (Uniprot-TrEMBL)
CSNK1D ProteinP48730 (Uniprot-TrEMBL)
CSNK1E ProteinP49674 (Uniprot-TrEMBL)
Centrosome:C2CD3:distal appendage proteins:TTBK2ComplexR-HSA-5626178 (Reactome)
Centrosome:C2CD3:distal appendage proteinsComplexR-HSA-5626176 (Reactome)
CoA-SHMetaboliteCHEBI:15346 (ChEBI)
DCTN1-2 ProteinQ14203-2 (Uniprot-TrEMBL)
DCTN2 ProteinQ13561 (Uniprot-TrEMBL)
DCTN3 ProteinO75935 (Uniprot-TrEMBL)
DYNC1H1 ProteinQ14204 (Uniprot-TrEMBL)
DYNC1I2 ProteinQ13409 (Uniprot-TrEMBL)
DYNC2H1 ProteinQ8NCM8 (Uniprot-TrEMBL)
DYNC2LI1 ProteinQ8TCX1 (Uniprot-TrEMBL)
DYNLL1 ProteinP63167 (Uniprot-TrEMBL)
DYNLL2 ProteinQ96FJ2 (Uniprot-TrEMBL)
DYNLRB1 ProteinQ9NP97 (Uniprot-TrEMBL)
DYNLRB2 ProteinQ8TF09 (Uniprot-TrEMBL)
EXOC1 ProteinQ9NV70 (Uniprot-TrEMBL)
EXOC2 ProteinQ96KP1 (Uniprot-TrEMBL)
EXOC3 ProteinO60645 (Uniprot-TrEMBL)
EXOC4 ProteinQ96A65 (Uniprot-TrEMBL)
EXOC5 ProteinO00471 (Uniprot-TrEMBL)
EXOC6 ProteinQ8TAG9 (Uniprot-TrEMBL)
EXOC7 ProteinQ9UPT5 (Uniprot-TrEMBL)
EXOC8 ProteinQ8IYI6 (Uniprot-TrEMBL)
FBF1 ProteinQ8TES7 (Uniprot-TrEMBL)
FBF1ProteinQ8TES7 (Uniprot-TrEMBL)
FGFR1OP ProteinO95684 (Uniprot-TrEMBL)
GBF1ProteinQ92538 (Uniprot-TrEMBL)
GDP MetaboliteCHEBI:17552 (ChEBI)
GDPMetaboliteCHEBI:17552 (ChEBI)
GTP MetaboliteCHEBI:15996 (ChEBI)
GTPMetaboliteCHEBI:15996 (ChEBI)
Golgi-derived vesicle R-ALL-5637956 (Reactome)
H2OMetaboliteCHEBI:15377 (ChEBI)
HAUS1 ProteinQ96CS2 (Uniprot-TrEMBL)
HAUS2 ProteinQ9NVX0 (Uniprot-TrEMBL)
HAUS3 ProteinQ68CZ6 (Uniprot-TrEMBL)
HAUS4 ProteinQ9H6D7 (Uniprot-TrEMBL)
HAUS5 ProteinO94927 (Uniprot-TrEMBL)
HAUS6 ProteinQ7Z4H7 (Uniprot-TrEMBL)
HAUS7 ProteinQ99871 (Uniprot-TrEMBL)
HAUS8 ProteinQ9BT25 (Uniprot-TrEMBL)
HDAC6ProteinQ9UBN7 (Uniprot-TrEMBL)
HSP90AA1 ProteinP07900 (Uniprot-TrEMBL)
HSPB11 ProteinQ9Y547 (Uniprot-TrEMBL)
HSPB11ProteinQ9Y547 (Uniprot-TrEMBL)
IFT AComplexR-HSA-5617795 (Reactome)
IFT AComplexR-HSA-5625350 (Reactome)
IFT B*ComplexR-HSA-5617798 (Reactome)
IFT BComplexR-HSA-5617799 (Reactome)
IFT BComplexR-HSA-5625418 (Reactome)
IFT122 ProteinQ9HBG6 (Uniprot-TrEMBL)
IFT122ProteinQ9HBG6 (Uniprot-TrEMBL)
IFT140 ProteinQ96RY7 (Uniprot-TrEMBL)
IFT140ProteinQ96RY7 (Uniprot-TrEMBL)
IFT172 ProteinQ9UG01 (Uniprot-TrEMBL)
IFT172ProteinQ9UG01 (Uniprot-TrEMBL)
IFT20 ProteinQ8IY31 (Uniprot-TrEMBL)
IFT20:TRIP11ComplexR-HSA-5617801 (Reactome)
IFT20ProteinQ8IY31 (Uniprot-TrEMBL)
IFT27 ProteinQ9BW83 (Uniprot-TrEMBL)
IFT27ProteinQ9BW83 (Uniprot-TrEMBL)
IFT43 ProteinQ96FT9 (Uniprot-TrEMBL)
IFT43ProteinQ96FT9 (Uniprot-TrEMBL)
IFT46 ProteinQ9NQC8 (Uniprot-TrEMBL)
IFT46ProteinQ9NQC8 (Uniprot-TrEMBL)
IFT52 ProteinQ9Y366 (Uniprot-TrEMBL)
IFT52ProteinQ9Y366 (Uniprot-TrEMBL)
IFT57 ProteinQ9NWB7 (Uniprot-TrEMBL)
IFT57ProteinQ9NWB7 (Uniprot-TrEMBL)
IFT74 ProteinQ96LB3 (Uniprot-TrEMBL)
IFT74ProteinQ96LB3 (Uniprot-TrEMBL)
IFT80 ProteinQ9P2H3 (Uniprot-TrEMBL)
IFT80ProteinQ9P2H3 (Uniprot-TrEMBL)
IFT81 ProteinQ8WYA0 (Uniprot-TrEMBL)
IFT81ProteinQ8WYA0 (Uniprot-TrEMBL)
IFT88 ProteinQ13099 (Uniprot-TrEMBL)
IFT88ProteinQ13099 (Uniprot-TrEMBL)
INPP5E ProteinQ9NRR6 (Uniprot-TrEMBL)
INPP5EProteinQ9NRR6 (Uniprot-TrEMBL)
IQCB1 ProteinQ15051 (Uniprot-TrEMBL)
KIF17 ProteinQ9P2E2 (Uniprot-TrEMBL)
KIF17 dimer:TNPO1ComplexR-HSA-5624928 (Reactome)
KIF17 dimer:TNPO1ComplexR-HSA-5624930 (Reactome)
KIF17 dimerComplexR-HSA-5624926 (Reactome)
KIF24 ProteinQ5T7B8 (Uniprot-TrEMBL)
KIF3A ProteinQ9Y496 (Uniprot-TrEMBL)
KIF3B ProteinO15066 (Uniprot-TrEMBL)
KIF3C ProteinO14782 (Uniprot-TrEMBL)
KIFAP3 ProteinQ92845 (Uniprot-TrEMBL)
Kinesin-2 motorsComplexR-HSA-5624910 (Reactome)
Kinesin-2 motorsComplexR-HSA-5625387 (Reactome)
LZTFL1 oligomer:BBSomeComplexR-HSA-5624121 (Reactome)
LZTFL1 ProteinQ9NQ48 (Uniprot-TrEMBL)
LZTFL1 oligomerComplexR-HSA-5624119 (Reactome)
MAPRE1 ProteinQ15691 (Uniprot-TrEMBL)
MARK4 ProteinQ96L34 (Uniprot-TrEMBL)
MARK4ProteinQ96L34 (Uniprot-TrEMBL)
MCHR1 ProteinQ99705 (Uniprot-TrEMBL)
MKKS ProteinQ9NPJ1 (Uniprot-TrEMBL)
MKS1 ProteinQ9NXB0 (Uniprot-TrEMBL)
Microtubule protofilament R-HSA-8982424 (Reactome)
MicrotubuleComplexR-HSA-190599 (Reactome)
MyrG2-CYS1 ProteinQ717R9 (Uniprot-TrEMBL)
MyrG2-NPHP3 ProteinQ7Z494 (Uniprot-TrEMBL)
NDE1 ProteinQ9NXR1 (Uniprot-TrEMBL)
NEDD1 ProteinQ8NHV4 (Uniprot-TrEMBL)
NEK2 ProteinP51955 (Uniprot-TrEMBL)
NINL ProteinQ9Y2I6 (Uniprot-TrEMBL)
NPHP complexComplexR-HSA-5626668 (Reactome)
NPHP1 ProteinO15259 (Uniprot-TrEMBL)
NPHP4 ProteinO75161 (Uniprot-TrEMBL)
ODF2 ProteinQ5BJF6 (Uniprot-TrEMBL)
OFD1 ProteinO75665 (Uniprot-TrEMBL)
PAFAH1B1 ProteinP43034 (Uniprot-TrEMBL)
PCM1 ProteinQ15154 (Uniprot-TrEMBL)
PCNT ProteinO95613 (Uniprot-TrEMBL)
PDE6D ProteinO43924 (Uniprot-TrEMBL)
PDE6D:INPP5EComplexR-HSA-5624934 (Reactome)
PDE6D:INPP5EComplexR-HSA-5624936 (Reactome)
PDE6DProteinO43924 (Uniprot-TrEMBL)
PKD1 ProteinP98161 (Uniprot-TrEMBL)
PKD2 ProteinQ13563 (Uniprot-TrEMBL)
PLK1 ProteinP53350 (Uniprot-TrEMBL)
PLK4 ProteinO00444 (Uniprot-TrEMBL)
PPP2R1A ProteinP30153 (Uniprot-TrEMBL)
PRKACA ProteinP17612 (Uniprot-TrEMBL)
PRKAR2B ProteinP31323 (Uniprot-TrEMBL)
PiMetaboliteCHEBI:43474 (ChEBI)
RAB11A ProteinP62491 (Uniprot-TrEMBL)
RAB11A:GTP:Golgi derived vesicleComplexR-HSA-5637979 (Reactome)
RAB11A:GTP:RAB11FIP3

dimer:ASAP1

dimer:ARF4:GTP:VxPx-containing ciliary membrane proteins		ComplexR-HSA-5623434 (Reactome)
RAB11A:GTP:RAB11FIP3

dimer:ASAP1

dimer:VxPx-containing ciliary membrane proteins		ComplexR-HSA-5623437 (Reactome)
RAB11A:GTPComplexR-HSA-5623431 (Reactome)
RAB11FIP3 ProteinO75154 (Uniprot-TrEMBL)
RAB11FIP3 dimerComplexR-HSA-5623435 (Reactome)
RAB3IP ProteinQ96QF0 (Uniprot-TrEMBL)
RAB3IP:BBSomeComplexR-HSA-5617806 (Reactome)
RAB3IP:RAB11A:GTP:Golgi-derived vesicleComplexR-HSA-5637982 (Reactome)
RAB3IP:RAB8A:GDPComplexR-HSA-5623440 (Reactome)
RAB3IPProteinQ96QF0 (Uniprot-TrEMBL)
RAB8A ProteinP61006 (Uniprot-TrEMBL)
RAB8A:GDP:RAB3IP:RAB11A:GTP:FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsComplexR-HSA-5623444 (Reactome)
RAB8A:GDPComplexR-HSA-5623441 (Reactome)
RAB8A:GTP:RAB3IP:RAB11A:GTP:RAB11FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsComplexR-HSA-5623447 (Reactome)
RAB8A:GTP:RAB3IP:RAB11A:GTP:RAB11FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsComplexR-HSA-5623450 (Reactome)
RAB8A:GTPComplexR-HSA-5623445 (Reactome)
RABL5 ProteinQ9H7X7 (Uniprot-TrEMBL)
RABL5ProteinQ9H7X7 (Uniprot-TrEMBL)
RHO ProteinP08100 (Uniprot-TrEMBL)
RP2 ProteinO75695 (Uniprot-TrEMBL)
RP2:ARL3:GDP:UNC119BComplexR-HSA-5637984 (Reactome)
RP2:ARL3:GTP:UNC119B(active)ComplexR-HSA-5637988 (Reactome)
RP2:ARL3:GTP:UNC119BComplexR-HSA-5637985 (Reactome)
RP2ProteinO75695 (Uniprot-TrEMBL)
RPGRIP1L ProteinQ68CZ1 (Uniprot-TrEMBL)
SCLT1 ProteinQ96NL6 (Uniprot-TrEMBL)
SCLT1ProteinQ96NL6 (Uniprot-TrEMBL)
SDCCAG8 ProteinQ86SQ7 (Uniprot-TrEMBL)
SEPT2ProteinQ15019 (Uniprot-TrEMBL)
SFI1 ProteinA8K8P3 (Uniprot-TrEMBL)
SMO ProteinQ99835 (Uniprot-TrEMBL)
SSNA1 ProteinO43805 (Uniprot-TrEMBL)
SSTR3 ProteinP32745 (Uniprot-TrEMBL)
TCP1 ProteinP17987 (Uniprot-TrEMBL)
TCTE3 ProteinQ8IZS6 (Uniprot-TrEMBL)
TCTEX1D1 ProteinQ8N7M0 (Uniprot-TrEMBL)
TCTEX1D2 ProteinQ8WW35 (Uniprot-TrEMBL)
TCTN1 ProteinQ2MV58 (Uniprot-TrEMBL)
TCTN2 ProteinQ96GX1 (Uniprot-TrEMBL)
TCTN3 ProteinQ6NUS6 (Uniprot-TrEMBL)
TMEM216 ProteinQ9P0N5 (Uniprot-TrEMBL)
TMEM67 ProteinQ5HYA8 (Uniprot-TrEMBL)
TNPO1 ProteinQ92973 (Uniprot-TrEMBL)
TNPO1ProteinQ92973 (Uniprot-TrEMBL)
TRAF3IP1 ProteinQ8TDR0 (Uniprot-TrEMBL)
TRAF3IP1ProteinQ8TDR0 (Uniprot-TrEMBL)
TRIP11 ProteinQ15643 (Uniprot-TrEMBL)
TRIP11ProteinQ15643 (Uniprot-TrEMBL)
TTBK2 ProteinQ6IQ55 (Uniprot-TrEMBL)
TTBK2ProteinQ6IQ55 (Uniprot-TrEMBL)
TTC21B ProteinQ7Z4L5 (Uniprot-TrEMBL)
TTC21BProteinQ7Z4L5 (Uniprot-TrEMBL)
TTC26 ProteinA0AVF1 (Uniprot-TrEMBL)
TTC26ProteinA0AVF1 (Uniprot-TrEMBL)
TTC30A ProteinQ86WT1 (Uniprot-TrEMBL)
TTC30B ProteinQ8N4P2 (Uniprot-TrEMBL)
TTC30ComplexR-HSA-5637976 (Reactome)
TTC8 ProteinQ8TAM2 (Uniprot-TrEMBL)
TTC8ProteinQ8TAM2 (Uniprot-TrEMBL)
TUBA1A ProteinQ71U36 (Uniprot-TrEMBL)
TUBA4A ProteinP68366 (Uniprot-TrEMBL)
TUBB ProteinP07437 (Uniprot-TrEMBL)
TUBB4A ProteinP04350 (Uniprot-TrEMBL)
TUBB4B ProteinP68371 (Uniprot-TrEMBL)
TUBG1 ProteinP23258 (Uniprot-TrEMBL)
Tectonic-like complexComplexR-HSA-5626670 (Reactome)
UNC119B ProteinA6NIH7 (Uniprot-TrEMBL)
UNC119B:myristoylated ciliary cargoComplexR-HSA-5624105 (Reactome)
UNC119B:myristoylated ciliary cargoComplexR-HSA-5624122 (Reactome)
UNC119BProteinA6NIH7 (Uniprot-TrEMBL)
VxPx-containing

ciliary membrane

proteins		ComplexR-HSA-5620919 (Reactome)
VxPx-containing

ciliary membrane

proteins		ComplexR-HSA-5623420 (Reactome)
WDR19 ProteinQ8NEZ3 (Uniprot-TrEMBL)
WDR19ProteinQ8NEZ3 (Uniprot-TrEMBL)
WDR34 ProteinQ96EX3 (Uniprot-TrEMBL)
WDR35 ProteinQ9P2L0 (Uniprot-TrEMBL)
WDR35ProteinQ9P2L0 (Uniprot-TrEMBL)
WDR60 ProteinQ8WVS4 (Uniprot-TrEMBL)
YWHAE ProteinP62258 (Uniprot-TrEMBL)
YWHAG ProteinP61981 (Uniprot-TrEMBL)
acetylated microtubuleComplexR-HSA-5618327 (Reactome)
acetylated microtubuleR-HSA-5624939 (Reactome)
acetylated microtubule R-HSA-5624939 (Reactome)
acetylated microtubule protofilament R-HSA-8982429 (Reactome)
active dynein-2 motorsComplexR-HSA-5625410 (Reactome)
active kinesin-2 motorsComplexR-HSA-5624943 (Reactome)
anterograde IFT trainsComplexR-HSA-5624945 (Reactome)
anterograde IFT trainsComplexR-HSA-5625417 (Reactome)
basal

body:transition zone

proteins:RAB3IP:RAB11A:GTP:Golgi-derived vesicle		ComplexR-HSA-5637989 (Reactome)
basal

body:transition

zone proteins		ComplexR-HSA-5626673 (Reactome)
basal bodyComplexR-HSA-5626181 (Reactome)
centrosome:C2CD2:distal appendage proteins:TTBK2:MARK4ComplexR-HSA-5626678 (Reactome)
dynein-2ComplexR-HSA-5624940 (Reactome)
dynein-2ComplexR-HSA-5625411 (Reactome)
exocyst complex:RAB8A:GTP:RAB3IP:RAB11:GTP:RAB11FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsComplexR-HSA-5623455 (Reactome)
exocyst complexComplexR-HSA-5623453 (Reactome)
mother centriole:C2CD3ComplexR-HSA-5626175 (Reactome)
mother centrioleComplexR-HSA-5626171 (Reactome)
myristoylated ciliary cargoComplexR-HSA-5624099 (Reactome)
myristoylated ciliary proteinsComplexR-HSA-5624102 (Reactome)
retrograde IFT trainsComplexR-HSA-5624947 (Reactome)
retrograde IFT trainsComplexR-HSA-5625425 (Reactome)

Annotated Interactions

View all...
SourceTargetTypeDatabase referenceComment
ARF4:GDPArrowR-HSA-5623513 (Reactome)
ARF4:GDPR-HSA-5623508 (Reactome)
ARF4:GTP:VxPx-containing ciliary membrane proteinsArrowR-HSA-5620914 (Reactome)
ARF4:GTP:VxPx-containing ciliary membrane proteinsR-HSA-5620918 (Reactome)
ARF4:GTPArrowR-HSA-5623508 (Reactome)
ARF4:GTPR-HSA-5620914 (Reactome)
ARL13B:INPP5E:PDE6DArrowR-HSA-5624953 (Reactome)
ARL13B:INPP5E:PDE6DR-HSA-5638012 (Reactome)
ARL13B:INPP5EArrowR-HSA-5638012 (Reactome)
ARL13BR-HSA-5624953 (Reactome)
ARL3:GDPArrowR-HSA-5638016 (Reactome)
ARL3:GTP:UNC119B:myristoylated ciliary cargoArrowR-HSA-5624133 (Reactome)
ARL3:GTP:UNC119B:myristoylated ciliary cargoR-HSA-5624130 (Reactome)
ARL3:GTP:UNC119BArrowR-HSA-5624130 (Reactome)
ARL3:GTP:UNC119BR-HSA-5638004 (Reactome)
ARL3:GTPR-HSA-5624133 (Reactome)
ARL6:GTP:BBSome:ciliary cargoArrowR-HSA-5624126 (Reactome)
ARL6:GTP:BBSome:ciliary cargoArrowR-HSA-5624127 (Reactome)
ARL6:GTP:BBSome:ciliary cargoR-HSA-5624127 (Reactome)
ARL6:GTPR-HSA-5624126 (Reactome)
ASAP1

dimer:

ARF4:GTP:VxPx-containing ciliary membrane proteins		ArrowR-HSA-5620918 (Reactome)
ASAP1

dimer:

ARF4:GTP:VxPx-containing ciliary membrane proteins		R-HSA-5620921 (Reactome)
ASAP1 dimerR-HSA-5620918 (Reactome)
ATATmim-catalysisR-HSA-5618328 (Reactome)
Ac-CoAArrowR-HSA-5618331 (Reactome)
Ac-CoAR-HSA-5618328 (Reactome)
BBIP1R-HSA-5624125 (Reactome)
BBS/CCT complexArrowR-HSA-5624125 (Reactome)
BBS1R-HSA-5624125 (Reactome)
BBS2R-HSA-5624125 (Reactome)
BBS4R-HSA-5624125 (Reactome)
BBS5R-HSA-5624125 (Reactome)
BBS7R-HSA-5624125 (Reactome)
BBS9R-HSA-5624125 (Reactome)
BBSome ciliary cargoR-HSA-5624126 (Reactome)
BBSomeArrowR-HSA-5624125 (Reactome)
BBSomeR-HSA-5617815 (Reactome)
BBSomeR-HSA-5624126 (Reactome)
BBSomeR-HSA-5624129 (Reactome)
C2CD3R-HSA-5626220 (Reactome)
CCP110ArrowR-HSA-5626227 (Reactome)
CEP164R-HSA-5626223 (Reactome)
CEP83R-HSA-5626223 (Reactome)
CEP89R-HSA-5626223 (Reactome)
CEP97ArrowR-HSA-5626227 (Reactome)
CLUAPR-HSA-5617820 (Reactome)
Centrosome:C2CD3:distal appendage proteins:TTBK2ArrowR-HSA-5626228 (Reactome)
Centrosome:C2CD3:distal appendage proteins:TTBK2R-HSA-5626699 (Reactome)
Centrosome:C2CD3:distal appendage proteinsArrowR-HSA-5626223 (Reactome)
Centrosome:C2CD3:distal appendage proteinsR-HSA-5626228 (Reactome)
CoA-SHArrowR-HSA-5618328 (Reactome)
CoA-SHR-HSA-5618331 (Reactome)
FBF1R-HSA-5626223 (Reactome)
GBF1mim-catalysisR-HSA-5623508 (Reactome)
GDPArrowR-HSA-5617816 (Reactome)
GDPArrowR-HSA-5623508 (Reactome)
GDPArrowR-HSA-5623521 (Reactome)
GTPR-HSA-5617816 (Reactome)
GTPR-HSA-5623508 (Reactome)
GTPR-HSA-5623521 (Reactome)
H2OR-HSA-5623513 (Reactome)
HDAC6mim-catalysisR-HSA-5618331 (Reactome)
HSPB11R-HSA-5617820 (Reactome)
IFT AArrowR-HSA-5617829 (Reactome)
IFT AArrowR-HSA-5625421 (Reactome)
IFT AArrowR-HSA-5625424 (Reactome)
IFT AR-HSA-5624949 (Reactome)
IFT AR-HSA-5624952 (Reactome)
IFT B*ArrowR-HSA-5617820 (Reactome)
IFT B*R-HSA-5617825 (Reactome)
IFT BArrowR-HSA-5617825 (Reactome)
IFT BArrowR-HSA-5625421 (Reactome)
IFT BArrowR-HSA-5625424 (Reactome)
IFT BR-HSA-5624949 (Reactome)
IFT BR-HSA-5624952 (Reactome)
IFT122R-HSA-5617829 (Reactome)
IFT140R-HSA-5617829 (Reactome)
IFT172R-HSA-5617820 (Reactome)
IFT20:TRIP11R-HSA-5617828 (Reactome)
IFT20ArrowR-HSA-5617828 (Reactome)
IFT20R-HSA-5617825 (Reactome)
IFT27R-HSA-5617820 (Reactome)
IFT43R-HSA-5617829 (Reactome)
IFT46R-HSA-5617820 (Reactome)
IFT52R-HSA-5617820 (Reactome)
IFT57R-HSA-5617820 (Reactome)
IFT74R-HSA-5617820 (Reactome)
IFT80R-HSA-5617820 (Reactome)
IFT81R-HSA-5617820 (Reactome)
IFT88R-HSA-5617820 (Reactome)
INPP5ER-HSA-5624956 (Reactome)
KIF17 dimer:TNPO1ArrowR-HSA-5624948 (Reactome)
KIF17 dimer:TNPO1ArrowR-HSA-5624951 (Reactome)
KIF17 dimer:TNPO1R-HSA-5624948 (Reactome)
KIF17 dimerR-HSA-5624951 (Reactome)
Kinesin-2 motorsArrowR-HSA-5625421 (Reactome)
Kinesin-2 motorsArrowR-HSA-5625424 (Reactome)
Kinesin-2 motorsR-HSA-5624952 (Reactome)
LZTFL1 oligomer:BBSomeArrowR-HSA-5624129 (Reactome)
LZTFL1 oligomer:BBSomeTBarR-HSA-5624127 (Reactome)
LZTFL1 oligomerR-HSA-5624129 (Reactome)
MARK4R-HSA-5626699 (Reactome)
MicrotubuleArrowR-HSA-5618331 (Reactome)
MicrotubuleR-HSA-5618328 (Reactome)
NPHP complexR-HSA-5626681 (Reactome)
PDE6D:INPP5EArrowR-HSA-5624954 (Reactome)
PDE6D:INPP5EArrowR-HSA-5624956 (Reactome)
PDE6D:INPP5ER-HSA-5624953 (Reactome)
PDE6D:INPP5ER-HSA-5624954 (Reactome)
PDE6DArrowR-HSA-5638012 (Reactome)
PDE6DR-HSA-5624956 (Reactome)
PiArrowR-HSA-5623513 (Reactome)
R-HSA-5617815 (Reactome) The BBS1 component of the BBSome complex binds RAB3IP, a GEF for the small GTPase RAB8A. RAB3IP is required for RAB8A to localize to the cilium, and depletion of RAB3IP compromises cilia formation (Nachury et al, 2007; Loktev et al, 2008). GTP-bound RAB8A may promote ciliogenesis by promoting the traffic of post-Golgi vesicles to the base of the cilium (Nachury et al, 2007; reviewed in Zerial and McBride, 2001; Ishikawa et al, 2011; Hsiao et al, 2012; Sung and Leroux, 2013).
R-HSA-5617816 (Reactome) RAB3IP is a GEF for RAB8A, the only RAB GTPase localized in the cilium. GTP-bound RAB8A may play a role in recruiting vesicles from the Golgi to the ciliary base and is required for cilia formation (Nachury et al, 2007; Yoshimura et al, 2007; reviewed in Reiter et al, 2012).
R-HSA-5617820 (Reactome) Based on studies done in C. reinhardtii, C. elegans and mouse, the human IFT B complex likely consists of, minimally, IFT20, RABL5/IFT22, HSPB11/IFT25, IFT27, IFT46, IFT52, TRAF3IP1/IFT54, IFT74, IFT80, IFT81, IFT88, CLUAP/QILIN, IFT70/TTC30, and TTC26/IFT56, with IFT172 being an additional candidate (Follit et al, 2009; Piperno and Mead, 1997; Cole et al, 1998; Cole, 2003; Ou, 2007; Hallbritter et al, 2013; reviewed in Taschner et al, 2012). Work in C. reinhardtii and mouse suggests that IFT B consists of a salt stable core complex of IFT88, IFT81, IFT74, IFT70, IFT52, IFT46 IFT 27, IFT25 and IFT22 with peripheral, weakly associated subunits IFT 172, IFT80, IFT57, CLUAP, TTC26 and IFT20 (Lucker et al, 2005; Lucker et al, 2010; Follit et al, 2009; Bhogaraju et al, 2011). In Chlamydomonas, core components IFT81 and IFT74 have been shown to interact directly and a stable sub-complex of IFT81/74/27/25 has been demonstrated (Lucker et al, 2005; Taschner et al, 2011). Human IFT81 and IFT74 have likewise been shown to directly interact and to form a tubulin-binding complex (Bhogaraju et al, 2013). A recent study has elucidated more detail of the protein-protein interactions that direct the assembly of the IFTB complex (Taschner et al, 2014).
This reaction shows putative human IFT B proteins assembling in a single step; details of how and when this assembly occurs are not shown, nor are the specific protein-protein interactions within the complex or details of how IFT B is regulated. Moreover, this reaction shows the formation of a presumptive IFT B* complex, lacking IFT20, to allow the recruitment of IFT20 from the Golgi compartment to be depicted.
R-HSA-5617825 (Reactome) IFT20 is unique among IFT B components in that, in addition to being localized at the cilium and the centrosome, a pool of IFT20 exists at the Golgi in complex with the golgin protein TRIP11 (Follit et al, 2006; Follit et al, 2008; Follit et al, 2009). Independent of its interaction with TRIP11, IFT20 has been shown to interact with the IFT B complex member TRAF3IP1 at the cilium, and overexpression of TRAF3IP1 displaces IFT20 from the Golgi (Follit et al, 2009). Partial depletion of IFT20 disrupts the traffic of membrane proteins to the cilium (Follit et al, 2006; Follit et al, 2009). Taken together, these data suggest a model where TRAF3IP1 mediates recruitment of IFT20-carrying vesicles from the Golgi to the site of cilium assembly, thus completing assembly of the IFT B complex and delivering both lipid and protein cargo for cilium biogenesis (Follit et al, 2008; Follit et al, 2009; reviewed in Ishikawa et al, 2011).
R-HSA-5617828 (Reactome) IFT20 is a member of the IFT B anterograde complex that is required for cilia formation and that, uniquely among IFT proteins, is found at the Golgi in addition to the centrosome and the cilium. Fluorescently-labelled IFT20 shuttles between the Golgi complex and the cilium and the ciliary microtubules (Follit et al, 2006; Follit et al, 2009). Golgi-association of IFT20 depends on interaction with the peripheral membrane protein TRIP11 and this interaction occurs independently of the IFT B complex (Follit et al, 2008). Golgi-localization of IFT20 is abolished in cells lacking TRIP11, and cilia in these cells are short and have a depleted complement of polycystin-2, a ciliary-localized membrane protein (Follit et al, 2008). RNAi-depletion of IFT20 in mammalian cells similarly compromises the traffic of polycystin-2 to the cilium (Follit et al, 2006). These data suggest that IFT20 may have a role at the Golgi complex in sorting and transporting membrane proteins that are destined for the cilium (Follit et al, 2006; Follit et al, 2008; Follit et al, 2009). IFT54, another IFT B component that is localized at the cilia, interacts with IFT20 but not with TRIP11, and overexpression of IFT54 displaces IFT20 from the Golgi. This supports a model where, after dissociation of the TRIP11:IFT20 complex, IFT54 docks IFT20 at the cilium, possibly on the surface of Golgi-derived vesicles, thus completing assembly of the IFT B complex and delivering ciliary membrane and membrane proteins to the site of cilium assembly (Follit et al, 2009; Omori et al, 2008; Li et al, 2008).
R-HSA-5617829 (Reactome) The IFT A complex is believed to be composed of six components: WDR19/IFT144, IFT140, IFT122, TTC21B/IFT139, WDR35/IFT121 and IFT43 (Piperno et al, 1998; Cole and Snell, 2009; reviewed in Taschner et al, 2012). Each of these proteins was identified as a TULP3-interacting protein in human cells, supporting the notion established in other organisms that they are all components of the IFT A complex (Mukhopadhyay et al, 2010; reviewed in Taschner et al, 2012). The IFT A proteins are large and generally have similar domain organization, consisting of N-terminal WD motifs and C-terminal TPR repeats. These protein interaction domains may help the IFT A complex scaffold recruitment of the IFT B complex, as well as recruit ciliary cargo and motor proteins. Intriguingly, the domain structure of IFT A proteins is similar to that of nucleoporins and coat proteins and it has been suggested that they evolved from a coat protein precursor, consistent with a role in vesicle trafficking (Devos et al, 2004; Jekely and Arendt, 2006).
Details of protein-protein interactions within the IFT A complex are not known, nor are the details of how and where the complex assembles in a human cell.
R-HSA-5618328 (Reactome) Cilia are enriched in acetylated tubulin, a marker that is associated with stability, and the kinesin motor preferentially travels on acetylated microtubules (Johnson et al, 1998; Reed et al, 2006; Cai et al, 2009). Alpha tubulin acetyltransferase (ATAT), also known as MEC17, has been shown to catalyze the acetylation of alpha tubulin at K40 (Akella et al, 2010; Shida et al, 2010; Montagnac et al, 2013). ATAT preferentially acetylates polymerized alpha tubulin and may access the luminal K40 residue through 'breathing' of the microtubules protofilaments (Shida et al, 2010). ATAT was identified as an interacting protein with components of the BBSome, and siRNA knockdown of ATAT delays assembly of the primary cilium (Jin et al, 2010; Shida et al, 2010). BBIP1 is another component of the BBSome that has been shown to affect tubulin acetylation and stability, potentially through its interaction with HDAC6. BBIP1 exerts its effect on microtubule acetylation independently of its role as a component of the BBSome; it may exert its indirect effect by promoting ATAT-mediated acceleration, by counteracting HDAC6-mediated deacetylation, or by another mechanism (Loktev et al, 2008)
R-HSA-5618331 (Reactome) HDAC6 is a microtubule-associated deacetylase that targets the K40 acetyl groups of alpha tubulin (Hubbert et al, 2002; Loktev et al, 2008; Zhang et al, 2008). HDAC6 also interacts with BBIP1, a component of the BBSome that is required for BBSome assembly, and additionally (and independently of its role in the BBSome) plays a role in microtubule polymerization and acetylation (Loktev et al, 2008). Depletion of BBIP1 causes a marked reduction in cytoplasmic microtubule acetylation, and this defect is partially overcome by inhibition of HDAC6. These data suggest that BBIP1 may exert its effect on microtubule acetylation by negatively regulating HDAC6, although other mechanisms are also possible (Loktev et al, 2008).
R-HSA-5620914 (Reactome) ARF4 is a small GTPase that faciliates the targeting of some membrane proteins destined for the cilium. ARF4-mediated targeting of these cargo may depend in part on a VxPx-like motif in the C-terminal tails (Deretic et al, 2005; Geng et al, 2006; Jenkins et al, 2006; Mazelova et al, 2009; Ward et al, 2011; Wang et al, 2012; reviewed in Li et al, 2012). ARF4 is the only ARF protein with a role in ciliogenesis, and its localization at the TGN positions it to act as primary sorting mechanism for membrane proteins destined for the cilium (reviewed in Deretic, 2013; Li et al, 2012). ARF4 is myristoylated at the N-terminus and is membrane-associated in its GTP-bound form (reviewed in Li et al, 2012).
R-HSA-5620918 (Reactome) ASAP1 is a dimeric ARF GTPase activating protein (GAP) and scaffolding protein that is recruited to the trans-Golgi network (TGN) through interactions with activated ARF4, PI(4,5)P2 and acidic phospholipids (Brown et al, 1998; Che et al, 2005; Nie et al, 2006). Once at the TGN, ASAP1 forms a tripartite complex with ARF4 and ciliary cargo, possibly by interacting with a putative C-terminal FR targeting motif present in a number of membrane proteins destined for the cilium, although this remains to be conclusively demonstrated (Corbit et al, 2005; Wang et al, 2012; reviewed in Bhogaraju et al, 2013). In addition to its role as an ARF GAP, ASAP1 also scaffolds the recruitment of a number of other proteins required for ciliary targeting, including RAB11 and the RAB11 effector FIP3 (Mazelova et al, 2009; Inoue et al, 2008; reviewed in Deretic 2013).
R-HSA-5620921 (Reactome) Recruitment of ASAP1 to the TGN facilitates the subsequent recruitment of both RAB11A and the RAB11 effector protein FIP3 to the ciliary targeting complex. RAB11FIP3 functions as a homodimer and can bind simultaneously to RAB11 and ARF4 through its C-terminal region (Inoue et al, 2008; Mazelova et al, 2009; Wang et al, 2012; Shiba et al, 2006; Eathiraj et al, 2006; Schonteich et al, 2007). RAB11FIP3 also interacts with the BAR domain of ASAP1 and in this way may play a role in stimulating the ARF GAP activity of ASAP1, promoting the inactivation ARF4 and its subsequent dissociation from the TGN (Inoue et al, 2008; reviewed in Deretic, 2013).
R-HSA-5623508 (Reactome) GBF1 promotes guanine nucleotide exchange on ARF4 at the Golgi membrane, activating the small GTPase and promoting its association with the Golgi membrane (Szul et al, 2007; reviewed in Deretic, 2013). Activated ARF4 at the trans-Golgi network may represent the initial sorting point for membrane proteins destined for the cilium (reviewed in Li et al, 2012).
R-HSA-5623513 (Reactome) Recruitment of RAB11FIP3 to the trans-Golgi network (TGN) simulates ASAP1 to activate the ARF4 GTPase activity, causing the hydrolysis of GTP and the release of ARF4:GDP from the Golgi membrane (Mazelova et al, 2009; Inoue et al, 2008; reviewed in Deretic, 2013).
R-HSA-5623519 (Reactome) RAB8A is another small GTPase that is required for ciliogenesis. RAB8A is recruited to the ciliary targeting complex at the trans-Golgi network (TGN) through interactions of the RAB8A guanine nucleotide exchange factor (GEF) RAB3IP (also known as RABIN8) with ASAP1 and RAB11 (Wang et al, 2012; Westlake et al, 2011; Feng et al, 2012; reviewed in Deretic, 2013). RAB8A is recruited in the inactive GDP bound form, and is activated at the TGN by RAB3IP in a RAB11A-dependent fashion (Hatulla et al, 2002; Knodler et al, 2010; Westlake et al, 2012; Wang et al, 2012; Feng et al, 2012).
R-HSA-5623521 (Reactome) Once recruited to the ciliary targeting complex, RAB3IP/RABIN8 stimulates nucleotide exchange on RAB8A. Activated RAB8A is required for ciliogenesis and plays a role in mediating vesicle docking at the basal body, providing both lipid and protein content to the emerging cilium (Hattula et al, 2002; Knodler et al, 2010; Nachury et al, 2007; Wang et al, 2012; Westlake et al, 2011; Yoshimura et al, 2007; reviewed in Deretic, 2013; Sung and Leroux, 2013).


R-HSA-5623524 (Reactome) RAB3IP interacts directly with the EXOC6/SEC15 component of the exocyst, recruiting this membrane-targeting complex to the Golgi-derived vesicles (Feng et al, 2012; reviewed in Deretic, 2013). The exocyst is an octameric complex with roles in tethering and fusion of secretory vesicles with target membranes. A number of the exocyst components have been shown to be localized to the ciliary base and/or to be required for ciliogenesis. Consistent with this, IQCB1/NPHP5, a component of the basal body, has been shown to interact with members of the exocyst complex (Wu et al, 2005; Rogers et al, 2004; Zuo et al, 2009; Feng et al, 2012; Sang et al, 2011; reviewed in Das and Guo, 2011; Heider and Munson, 2012).
R-HSA-5623525 (Reactome) Membrane budding at the trans-Golgi network is promoted at least in part by the BAR domain of ASAP1, which is involved in sensing and inducing membrane curvature as well as providing the recognition site for small GTPases (Nie et al, 2006; Jian et al, 2009; Inoue et al, 2008; reviewed in Masuda et al, 2010). Oligomerization between ASAP1 and RAB11FIP3 may contribute to coat formation on vesicles budding from the TGN and destined for the plasma or ciliary membrane (Inoue et al, 2008; Mazelova et al, 2009; reviewed in Deretic, 2013).
R-HSA-5623527 (Reactome) Exocyst-mediated fusion of the Golgi-derived vesicle delivers the VxPx-containing membrane proteins to the ciliary membrane, although the precise mechanisms remain to be worked out (Mazelova et al, 2009; Wang et al, 2012; reviewed in Sung and Leroux, 2013). Vesicles carrying membrane proteins destined for the cilum may fuse at the periciliary membrane at the base of the cilium and deliver cargo to the IFT system. Ciliary membrane proteins may also diffuse laterally into the periciliary membrane after fusion of vesicles with the plasma membrane (reviewed in Hsiao et al, 2012; Sung and Leroux, 2013). Although not depicted in this reaction, there is evidence that some of the protein-protein interactions of the ciliary-targeting complex may persist into the periciliary or ciliary membrane region (Wang et al, 2012).
R-HSA-5624125 (Reactome) The BBSome is a complex of 8 conserved proteins with roles in ciliary trafficking (Nachury et al, 2007; Loktev et al, 2008; reviewed in Nachury et al, 2010; Hsiao et al, 2012). Mutations in the BBS genes leads to Bardet-Biedl syndrome, a heterogeneous ciliopathy characterized by obesity, blindness, cystic kidney disease, retinitis pigmentosa, polydactyly, mental retardation, and renal failure in some cases (reviewed in Tobin and Beales, 2009). The BBSome is the primary effector of ARL6/BBS3, a small GTPase that recruits the BBSome and associated membrane proteins destined for the cilium to membranes (Jin et al, 2010; Nachury et al, 2007; Zhang et al, 2011; Seo et al 2011). The BBSome also interacts with the RAB8A guanine nucleotide exchange factor RAB3IP, and in this way promotes the recruitment of RAB8A to the cilium (Nachury et al, 2007). Components of the BBSome are enriched in beta propeller and TPR domains and have been shown to form linear arrays on liposomes (Jin et al, 2010). Where these arrays form, and how they contribute to ciliary targeting remains to be elucidated (Jin et al, 2010; reviewed in Nachury et al, 2010).

In mammalian cells, formation of the BBSome depends on a BBS/CCT complex that consists of MKKS/BBS6, BBS10, BBS12 and 6 members of the CCT/TRiC family of chaperonins. The BBS/CCT complex interacts with a subset of the BBSome protein and plays a role in the BBS7 stability, promoting the formation of an intermediate "BBSome core complex" (Seo et al, 2010; Jin et al, 2010; Zhang el al, 2012).
R-HSA-5624126 (Reactome) ARL6 is a small GTPase that was also identified as BBS3, a gene that when mutated gives rise to the ciliopathy Bardet-Biedel syndrome (Chiang et al, 2004; Fan et al, 2004). In its GTP-form, membrane-associated ARL6 recruits the BBSome along with BBSome-associated cargo such as SSTR3, MHCR1 or SMO to the cilium (Jin et al, 2010; Zhang et al, 2011; Seo et al, 2011). Binding of IFT27 to the nucleotide-free form of ARL6 may also play a role in promoting the exit of the BBSome from the cilium (Liew et al, 2014). The BBSome, a complex consisting of BBS1, BBS2, BBS4, BBS5, BBS7, BBC9, TTC8/BBS8 and BBIP10 is thought to contribute to ciliary targeting, either by promoting budding of vesicles from the secretory pathway or through lateral diffusion of BBSome-enriched 'rafts' from the plasma membrane as indicated in this reaction (Jin et al, 2010; reviewed in Li et al, 2012; Sung and Leroux, 2013; Nachury et al, 2010). The interaction between the BBSome and ARL6 is mediated by the N-terminal B-propeller domain of BBSome component BBS1 (Jin et al, 2010). BBSome function is negatively regulated by LZTFL1, which forms a complex with the BBSome in the cytosol and inhibits its traffic to the cilium (Seo et al, 2011).
R-HSA-5624127 (Reactome) ARL6:GTP and the BBSome complex are required for the ciliary accumulation of proteins such as SSTR3, MHRC1 and SMO (Zhang et al, 2011; Jin et al, 2010; Seo et al, 2011; reviewed in Nachury et al, 2010; Sung and Leroux, 2013). BBSome localization to the primary cilium is negatively regulated by LZTFL1, and ciliary accumulation of some BBSome cargo is increased by LZTFL1 depletion (Seo et al, 2011).
R-HSA-5624129 (Reactome) LZTFL1 was identified as a tumor suppressor and as a protein that interacts with components of the BBSome (Wei et al, 2010; Seo et al, 2011). LZTFL1 forms cytosolic complexes with the BBSome and negatively regulates its entry into the cilium without affecting the assembly or stability of the BBSome complex. Both the BBSome and LZTFL1 have been shown to regulate the localization of the Hh signaling protein SMO (Seo et al, 2011). A recent study suggests that LZTFL1 may additionally play a role in coordinating the interaction between the BBSome and the IFT B component IFT27 and in this way contribute to the traffic of Hh pathway proteins into and out of the cilium (Eguether et al, 2014).
R-HSA-5624130 (Reactome) Binding of ARL3 to UNC119B induces a conformational change that obstructs UNC119B cargo-binding and promotes the release of the myrisoylated cargo into the ciliary membrane (Wright et al, 2011; Ismail et al, 2012).
R-HSA-5624131 (Reactome) UNC119B is an ARL3 effector that binds directly to the myristoyl moieties at glycine 2 of NPHP3 and CYS1 (Wright et al, 2011). Myristoylation is required for the ciliary localization of these proteins (Wright et al, 2011; Tao et al, 2006), and both mutation of the glycine 2 myristoylation target in NPHP3 and siRNA knockdown of UNC119B dramatically reduce the ciliary localization of NPHP3 and CYS1 (Tao et al, 2006; Wright et al, 2011; reviewed in Schwarz et al, 2012).
R-HSA-5624132 (Reactome) UNC119B promotes the translocation of myristoylated NPHP3 from the ER membrane to the cilium by an unknown mechanism. Ciliary localization depends both on myristoylation and UNC119B, as mutation of the glycine 2 acceptor site or siRNA knockdown of UNC119B drastically reduces the amount of NPHP3 or CYS1 in the cilium (Wright et al, 2011).
R-HSA-5624133 (Reactome) ARL3 is an ARF-like small GTPase that is localized to the cilium in both the GDP- and the GTP-bound form (Zhou et al, 2006; Wright et al, 2011). ARL3 binds UNC119B in a GTP-dependent fashion and is required for the ciliary localization of NPHP3 and CYS1. Upon GTPase activation, ARL3 promotes the transfer of the myristoylated cargo into the ciliary membrane (Wright et al, 2011).
R-HSA-5624948 (Reactome) Ciliary localization of the alternative kinesin-2 motor KIF17 depends on TNPO1 and the RAN:GDP/RAN:GTP gradient. Once in the cilium, the TNPO1:KIF17 complex is likely dissociated by RAN:GTP binding and subsequent GTP hydrolysis, freeing KIF17 to play its role in anterograde IFT transport (Dishinger et al, 2010).
R-HSA-5624949 (Reactome) IFT particles were first characterized in Chlamydomonas reinhardtii, where they were observed by differential interference contrast microscopy as electron-dense granules that move along doublet microtubules of the ciliary axoneme (Kozminski et al, 1993; Kozminski et al, 1995; reviewed in Pedersen et al, 2008). More recent ultrastructural analysis of Chlamydomonas flagella confirms the presence of two distinct types of IFT trains, a longer, less electron-opaque anterograde train and shorter, more opaque retrograde trains. Both the anterograde and retrograde trains are associated with the outer microtubule doublets and with the inner surface of the flagellar membrane (Pigino et al, 2009). Isolation and characterization of IFT particles revealed that they consist of 2 biochemically distinct subcomplexes, IFT A and IFT B that are widely conserved in ciliated organisms (Piperno et al, 1997; Cole et al, 1998; reviewed in Sung and Leroux, 2013). Anterograde traffic is driven by kinesin-2 type motors in an ATP-dependent manner. Evidence from C. elegans suggests distinct and sequential roles for the canonical heterotrimeric kinesin-2 motor and the alternate homodimeric kinesin-2, OSM-3 (homologue of human KIF17) in mediating anterograde transport, but this has not been demonstrated in human cells where the canonical kinesin-2 motor predominates (Evans et al, 2006; Snow et al, 2004; Ou et al, 2005). Human KIF17 appears to be required in some cell types for cilia formation, and plays a role in the import of some ciliary cargo (Jenkins et al, 2006; Insinna et al, 2008; Insinna et al, 2009; Dishinger et al, 2010; reviewed in Verhey et al, 2011). Assembly of the anterograde IFT trains at the base of the cilium may be facilitated by the BBSome complex, which has also been shown to display IFT-like movement along the axoneme; however, the BBSome is highly sub-stoichiometric with respect to the IFT complex, so this notion requires more substantiation (Ou et al, 2005; Wei et al, 2012; Blacque et al, 2004; Nachury et al, 2007; Lechtreck et al, 2009; reviewed in Sung and Leroux, 2013). Studies in C. elegans also suggest a role for ARL13B and ARL3 in regulating the stability of the anterograde IFT train (Li et al, 2010).
R-HSA-5624951 (Reactome) KIF17 is an alternate kinesin-2 motor that is required in some cell types for anterograde IFT and for the ciliary localization of CNG (Ou et al, 2005; Jenkins et al, 2006; Insinna et al, 2008; Insinna et al, 2009; Li et al, 2010; reviewed in Scholey, 2008; Verhey et al, 2011). KIF17 contains a C-terminal ciliary localization signal that mediates its interaction with nuclear import factor TNPO1 (importin beta-2). This interaction is required for the ciliary targeting of KIF17 and is regulated by RAN GTP levels such that the interaction is promoted in the cytosol where RAN:GTP levels are low, and is destabilized in the cilium where RAN:GTP levels are high (Dishinger et al, 2010). The roles of TNPO1 and the RAN:GTP gradient in promoting ciliary localization of KIF17 are analogous to their roles in nuclear import and provide evidence for the first time of conserved mechanisms governing nuclear and ciliary localization (Dishinger et al, 2010; Devos et al, 2004; Gruss, 2010).
R-HSA-5624952 (Reactome) Remodelling of IFT trains is thought to occur at the ciliary tip (Iomini et al, 2001; Buisson et al, 2013; reviewed in Snell and Cole, 2009). Retrograde transport is driven by the multi-subunit dynein-2 motor in an ATP-dependent fashion (Hou et al, 2004; Pazour et al, 1999; Porter et al, 1999; reviewed in Cole and Snell, 2009; Ishikawa et al, 2011). Mutations in genes encoding members of the IFT A complex or the dynein-2 motor generally result in short, swollen cilia that abnormally acccumulate IFT components (Iomini et al, 2009; Piperno et al, 1998; Pazour et al, 1999). The subunit composition of the human dynein-2 complex has recently been analyzed and preliminary characterization of the IFT A complex has begun, but detailed understanding of the molecular architecture of the retrograde IFT trains is still lacking (Assante et al, 2014; Piperno et al, 1998; Mukhopadhyay et al, 2010; reviewed in Taschner et al, 2012).
R-HSA-5624953 (Reactome) The small GTPase ARL13B is required for the ciliary localization of INPP5E. ARL13B binds directly to INPP5E and is thought to displace PDE6D from the complex (Humbert et al, 2012).
R-HSA-5624954 (Reactome) INPP5E is a ciliary peripheral membrane protein that is associated with the ciliopathy Joubert's Syndrome (Bielas et al, 2009; Jacoby et al, 2009). Ciliary localization of the full-length protein depends on a targeting sequence and interactions with PDE6D and ARL13B, although the detailed mechanism remains unresolved (Humbert et al, 2012).
R-HSA-5624956 (Reactome) The inositol polyphosphate phosphatase INPP5E is a ciliary localized peripheral membrane protein with a CaaX prenylation motif in its C-terminus (Jacoby et al, 2009; Bielas et al, 2009; Humbert et al, 2012). This motif is downstream of the ciliary targeting sequence and prenylation is not required for the ciliary localization of INPP5E. The CaaX motif is required for the interaction between INPP5E and the phosphodiesterase PDE6D, and PDE6D is required for the ciliary localization of full length INPP5E but not a truncated solubilized form. These data suggest that PDE6D may play a role in extracting prenylated INPP5E from a donor membrane prior to ciliary targeting (Humbert et al, 2012).
R-HSA-5625416 (Reactome) Anterograde trains travel along the axoneme of the cilium at an estimated rate of 2 micrometers per second in an ATP- and kinesin-2-dependent fashion (reviewed in Cole and Snell, 2009). Although the particulars of IFT train-cargo interactions have not been fully elaborated, recent studies in C. reinhardtii and human cells have shown that the IFT B components IFT74 and IFT81 have tubulin-binding sites, while IFT46 is required for the ciliary transport of the outer dynein arm, and more recently, TTC26 has been shown to be required for the transport of motility-related proteins into the flagella (Bhogaraju et al, 2013; Ahmed et al, 2008; Hou et al, 2007; Ishikawa et al, 2014; reviewed in Bhogarju et al, 2014).
R-HSA-5625421 (Reactome) Based on work done in C. reinhardtii and Trypanosoma brucei, anterograde IFT trains are believed to disassemble at the ciliary tip, releasing cargo and the IFT motors. Smaller retrograde trains are subsequently reassembled for transport back to the ciliary base (Iomini et al, 2001; Buisson et al, 2013; Pigino et al, 2009; reviewed in Ishikawa et al, 2011; Bhogaraju et al, 2013). A direct interaction between IFT27 and the nucleotide-free form of ARL6 may contribute to ARL6 activation and in this way contribute to ciliary exit of some cargo (Liew et al, 2014).
R-HSA-5625424 (Reactome) At the base of the cilium, retrograde trains are believed to disassemble and recycle for a subsequent round of IFT transport (Iomini et al, 2001; Buisson et al, 2013; reviewed in Ishikawa et al, 2011; Cole and Snell, 2009).
R-HSA-5625426 (Reactome) Retrograde trains are shorter than anterograde particles and travel along the cilliary axoneme at an estimated rate of 3 micrometers per second (reviewed in Cole and Snell, 2009).
R-HSA-5626220 (Reactome) C2 domain-containing protein 3 (C2CD3) is basal body-localized protein that is required for ciliogenesis and Hh signaling (Hoover et al, 2008; Balestra et al, 2013). C2CD3 is recruited to the centrosome in a pericentriolar material 1 protein (PCM1)- and microtubule-dependent manner and is required for the subsequent recruitment of the distal appendage proteins to the mother centriole (Tanos et al, 2013; Sillibourne et al, 2013; Ye et al, 2014; reviewed in Winey and O'Toole, 2014). The distal appendage proteins are thought to be a component of the transition fibres that anchor the basal body to the membrane at the base of the cilium and are themselves required for the recruitment of Tau-tubulin kinase 2 (TTBK2) and for docking ciliary vesicles to the mother centriole (Tanos et al, 2013; Wei et al, 2013; Joo et al, 2013; Schmidt et al, 2012; Sillibourne et al, 2013; Burke et al, 2014).
R-HSA-5626223 (Reactome) C2CD3 and the centrosome component OFD1 are required for the recruitment of distal appendage proteins CEP164, CEP83/CCDC41, CEP89, FBF1 and SCLT1 to the mother centriole (Ye et al, 2014; Tang et al, 2013; Singla et al, 2010). Distal appendage proteins are believed to form part of the transition fibres that anchor the basal bodies to the actin rich cortex at the base of the emerging cilium, and are also required for the recruitment and binding of ciliary vesicles and intraflagellar transport (IFT) complexes (Tanos et al, 2013; Wei et al, 2013; Joo et al, 2010; Schmidt et al, 2012; Sillibourne et al, 2013; Ye et al, 2014; reviewed in Winey and O'Toole, 2014).
R-HSA-5626227 (Reactome) CCP110 is a negative regulator of ciliogenesis that caps the mother centriole. CCP110 may inhibit ciliogenesis in part by preventing the CEP290-dependent recruitment of RAB8A to the centrosome and cilia (Spektor et al, 2007; Tsang et al, 2008; reviewed in Tsang and Dynlacht, 2013). CCP110 and CEP97 also form a complex with the KIF24, a kinesin with centriolar microtubule depolymerizing activity that is required for the initial recruitment and/or stability of CCP110 at the centriole (Kobayashi et al, 2011). Recruitment of TTBK2 promotes the displacement of CCP110 and its binding partner CEP97. This results in the formation of a basal body and promotes recruitment of IFT complex members and allows axonemal extension to occur (Goetz et al, 2012; Ye et al, 2014; reviewed in Tsang and Dynlacht, 2013). Although the kinase activity of TTBK2 is required for cilium formation and TTBK2 has been shown to phosphorylate CEP164, the relevant physiological target during ciliogenesis has not been unambiguously identified (Cajanek et al, 2014). Similarly, the kinase activity of MARK4 is also required for ciliogenesis, and the interaction between MARK4 and ODF2, a putative substrate, is needed to promote the dissociation of the CCP110 and CEP97 proteins from the centriole (Kuhns et al, 2013; reviewed in Kim and Dynlacht, 2013).
R-HSA-5626228 (Reactome) C2CD3 and the distal appendage protein CEP164 are required for the recruitment of the kinase Tau tubulin kinase 2 (TTBK2) to the centriole (Ye et al, 2014; Cajanek et al, 2014). TTBK2 recruitment promotes the release of CCP110, a negative regulator of ciliogenesis that caps the mother centriole (Goetz et al, 2012; Ye et al, 2014; Tsang et al, 2008; Spektor et al, 2007). CCP110 is initially recruited and/or stabilized at the mother centriole in a KIF24-dependent manner; KIF24, a kinesin-like protein, also restricts ciliogenesis through its microtubule depolymerizing activity (Kobayashi et al, 2011). In addition to promoting the release of CCP110, TTBK2 also plays a role in the recruitment of intraflagellar transport (IFT) proteins and in this way contributes to extension of the ciliary axoneme (Goetz et al, 2012; Ye et al, 2014). Mutations in TTBK2 disrupt ciliogenesis and are associated with the development of spinocerebellar ataxia (Houlden et al, 2007; Bouskila et al, 2011; reviewed in Jackson, 2012).
R-HSA-5626681 (Reactome) The transition zone is a protein-rich zone at the base of the cilium that forms after maturation of the mother centriole and prior to or concurrent with the initiation of intraflagellar transport (IFT) (reviewed in Benzing and Schermer, 2011; Reiter et al, 2012). The transition zone consists of a growing number of proteins and protein complexes, many of whose genes are associated with ciliopathies such as nephronophthisis, Meckel-Gruber syndrome and Joubert's syndrome (Reiter et al, 2006; Hu et al, 2010; Sang et al, 2011; Williams et al, 2011; Garcia-Gonzalo et al, 2011; Chih et al, 2012; reviewed in Reiter et al, 2012). In conjunction with SEPT2, which was recently shown to form a septin ring diffusion barrier at the base of the cilium, the transition zone and its resident proteins contribute to protein sorting and ciliary membrane composition and act as a ciliary gate (Hu et al, 2010; Williams et al, 2011; Garcia-Gonzalo et al, 2011; Chih et al, 2012; reviewed in Reiter et al, 2012).
R-HSA-5626699 (Reactome) MARK4 (microtubule associated protein/microtubule affinity regulating kinase 4) was identified in a screen as a positive regulator of ciliogenesis (Kuhns et al, 2013). MARK4 interacts at the centriole with the subdistal appendage component ODF2 and this interaction is required to promote axonemal extension (Kuhns et al, 2013; reviewed in Kim and Dynlacht, 2013). Like Tau tubulin kinase 2 (TTBK2), MARK4 appears to have a role in promoting the dissociation of CCP110 and CEP97, uncapping the centriole and allowing axonemal extension to take place (Kuhns et al, 2013; Ye et al, 2014;; Goetz et al, 2012). Although this reaction shows that MARK4 is recruited to the centriole subsequent to TTBK2, the timing of the ODF2:MARK4 interaction is not known.
R-HSA-5638004 (Reactome) RP2 is an ARL3 GAP that is localized to the cilium and plays a role in trafficking proteins from the Golgi to the ciliary membrane (Veltel et al, 2008a; Hurd et al, 2011; Evans et al, 2010; Wright et al, 2011). RP2 forms a ternary complex with UNC119B and ARL3, activating the ARL3 GTPase activity and promoting the release of UNC119B (Veltel et al, 2008b; Wright et al, 2011; Kuhnel et al, 2006; reviewed in Schwarz et al, 2012; Li et al, 2012)
R-HSA-5638006 (Reactome) RP2 is an ARL3 GAP that is localized to the cilium and plays a role in trafficking proteins from the Golgi to the ciliary membrane (Veltel et al, 2008a; Hurd et al, 2011; Evans et al, 2010; Wright et al, 2011). RP2 forms a ternary complex with UNC119B and ARL3, activating the ARL3 GTPase activity and promoting the release of UNC119B (Veltel et al, 2008b; Wright et al, 2011; Kuhnel et al, 2006; reviewed in Schwarz et al, 2012; Li et al, 2012)
R-HSA-5638007 (Reactome) RP2 is an ARL3 GAP that is localized to the cilium and plays a role in trafficking proteins from the Golgi to the ciliary membrane (Veltel et al, 2008a; Hurd et al, 2011; Evans et al, 2010; Wright et al, 2011). RP2 forms a ternary complex with UNC119B and ARL3, activating the ARL3 GTPase activity and promoting the release of UNC119B (Veltel et al, 2008b; Wright et al, 2011; Kuhnel et al, 2006; reviewed in Schwarz et al, 2012; Li et al, 2012)
R-HSA-5638009 (Reactome) The distal appendage protein CEP164 interacts with RAB3IP, and in this way recruits Golgi-derived vesicles to the basal body to initiate ciliary membrane biogenesis (Schmidt et al, 2012; Westlake et al, 2011; Rohatgi and Snell, 2010). RAB3IP recruitment to the distal appendages of centrioles promotes the appropriate localization and activation of RAB8A (Nachury et al, 2007; Yoshimura et al, 2007; Westlake et al, 2011; Schmidt et al, 2012).
R-HSA-5638012 (Reactome) ARL13B promotes the ciliary localization of INPP5E by interacting with it directly and displacing PDE6D from the complex (Humbert et al, 2012).
R-HSA-5638014 (Reactome) The RAB8 guanine nucleotide exchange factor RAB3IP/RABIN8 is recruited to vesicles through interaction with membrane-tethered RAB11:GTP (Westlake et al, 2011; Knodler et al, 2010). Recruitment of RAB3IP may also depend on the TRAPPCII complex, a multiprotein complex with roles in vesicular trafficking (Westlake et al, 2011; reviewed in Sacher et al, 2008). RAB3IP is required for RAB8A to localize to the cilium, and depletion of RAB3IP compromises cilia formation (Nachury et al, 2007; Loktev et al, 2008). GTP-bound RAB8A may promote ciliogenesis by promoting the traffic of post-Golgi vesicles to the base of the cilium (Nachury et al, 2007; Westlake et al, 2011; Feng et al, 2012; reviewed in Reiter et al, 2012)
R-HSA-5638016 (Reactome) ARL3 hydrolysis of GTP promotes the release of UNC119B, dissociating the complex (Wright et al, 2011; reveiwed in Schwarz et al, 2012).
RAB11A:GTP:Golgi derived vesicleR-HSA-5638014 (Reactome)
RAB11A:GTP:RAB11FIP3

dimer:ASAP1

dimer:ARF4:GTP:VxPx-containing ciliary membrane proteins		ArrowR-HSA-5620921 (Reactome)
RAB11A:GTP:RAB11FIP3

dimer:ASAP1

dimer:ARF4:GTP:VxPx-containing ciliary membrane proteins		R-HSA-5623513 (Reactome)
RAB11A:GTP:RAB11FIP3

dimer:ASAP1

dimer:ARF4:GTP:VxPx-containing ciliary membrane proteins		mim-catalysisR-HSA-5623513 (Reactome)
RAB11A:GTP:RAB11FIP3

dimer:ASAP1

dimer:VxPx-containing ciliary membrane proteins		ArrowR-HSA-5623513 (Reactome)
RAB11A:GTP:RAB11FIP3

dimer:ASAP1

dimer:VxPx-containing ciliary membrane proteins		R-HSA-5623519 (Reactome)
RAB11A:GTPR-HSA-5620921 (Reactome)
RAB11FIP3 dimerR-HSA-5620921 (Reactome)
RAB3IP:BBSomeArrowR-HSA-5617815 (Reactome)
RAB3IP:RAB11A:GTP:Golgi-derived vesicleArrowR-HSA-5638014 (Reactome)
RAB3IP:RAB11A:GTP:Golgi-derived vesicleR-HSA-5638009 (Reactome)
RAB3IP:RAB8A:GDPR-HSA-5623519 (Reactome)
RAB3IPR-HSA-5617815 (Reactome)
RAB3IPR-HSA-5638014 (Reactome)
RAB8A:GDP:RAB3IP:RAB11A:GTP:FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsArrowR-HSA-5623519 (Reactome)
RAB8A:GDP:RAB3IP:RAB11A:GTP:FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsR-HSA-5623521 (Reactome)
RAB8A:GDP:RAB3IP:RAB11A:GTP:FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsmim-catalysisR-HSA-5623521 (Reactome)
RAB8A:GDPR-HSA-5617816 (Reactome)
RAB8A:GTP:RAB3IP:RAB11A:GTP:RAB11FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsArrowR-HSA-5623521 (Reactome)
RAB8A:GTP:RAB3IP:RAB11A:GTP:RAB11FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsArrowR-HSA-5623525 (Reactome)
RAB8A:GTP:RAB3IP:RAB11A:GTP:RAB11FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsR-HSA-5623524 (Reactome)
RAB8A:GTP:RAB3IP:RAB11A:GTP:RAB11FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsR-HSA-5623525 (Reactome)
RAB8A:GTPArrowR-HSA-5617816 (Reactome)
RABL5R-HSA-5617820 (Reactome)
RP2:ARL3:GDP:UNC119BArrowR-HSA-5638006 (Reactome)
RP2:ARL3:GDP:UNC119BR-HSA-5638016 (Reactome)
RP2:ARL3:GTP:UNC119B(active)ArrowR-HSA-5638007 (Reactome)
RP2:ARL3:GTP:UNC119B(active)R-HSA-5638006 (Reactome)
RP2:ARL3:GTP:UNC119B(active)mim-catalysisR-HSA-5638006 (Reactome)
RP2:ARL3:GTP:UNC119BArrowR-HSA-5638004 (Reactome)
RP2:ARL3:GTP:UNC119BR-HSA-5638007 (Reactome)
RP2ArrowR-HSA-5638016 (Reactome)
RP2R-HSA-5638004 (Reactome)
RP2mim-catalysisR-HSA-5638007 (Reactome)
SCLT1R-HSA-5626223 (Reactome)
SEPT2ArrowR-HSA-5626681 (Reactome)
TNPO1R-HSA-5624951 (Reactome)
TRAF3IP1R-HSA-5617820 (Reactome)
TRIP11ArrowR-HSA-5617828 (Reactome)
TTBK2R-HSA-5626228 (Reactome)
TTC21BR-HSA-5617829 (Reactome)
TTC26R-HSA-5617820 (Reactome)
TTC30R-HSA-5617820 (Reactome)
TTC8R-HSA-5624125 (Reactome)
Tectonic-like complexR-HSA-5626681 (Reactome)
UNC119B:myristoylated ciliary cargoArrowR-HSA-5624131 (Reactome)
UNC119B:myristoylated ciliary cargoArrowR-HSA-5624132 (Reactome)
UNC119B:myristoylated ciliary cargoR-HSA-5624132 (Reactome)
UNC119B:myristoylated ciliary cargoR-HSA-5624133 (Reactome)
UNC119BArrowR-HSA-5638016 (Reactome)
UNC119BR-HSA-5624131 (Reactome)
VxPx-containing

ciliary membrane

proteins		ArrowR-HSA-5623527 (Reactome)
VxPx-containing

ciliary membrane

proteins		R-HSA-5620914 (Reactome)
WDR19R-HSA-5617829 (Reactome)
WDR35R-HSA-5617829 (Reactome)
acetylated microtubuleArrowR-HSA-5618328 (Reactome)
acetylated microtubuleArrowR-HSA-5625421 (Reactome)
acetylated microtubuleArrowR-HSA-5625424 (Reactome)
acetylated microtubuleR-HSA-5618331 (Reactome)
active dynein-2 motorsR-HSA-5624952 (Reactome)
active kinesin-2 motorsR-HSA-5624949 (Reactome)
anterograde IFT trainsArrowR-HSA-5624949 (Reactome)
anterograde IFT trainsArrowR-HSA-5625416 (Reactome)
anterograde IFT trainsR-HSA-5625416 (Reactome)
anterograde IFT trainsR-HSA-5625421 (Reactome)
basal

body:transition zone

proteins:RAB3IP:RAB11A:GTP:Golgi-derived vesicle		ArrowR-HSA-5638009 (Reactome)
basal

body:transition zone

proteins:RAB3IP:RAB11A:GTP:Golgi-derived vesicle		mim-catalysisR-HSA-5617816 (Reactome)
basal

body:transition

zone proteins		ArrowR-HSA-5626681 (Reactome)
basal

body:transition

zone proteins		R-HSA-5638009 (Reactome)
basal bodyArrowR-HSA-5626227 (Reactome)
basal bodyR-HSA-5626681 (Reactome)
centrosome:C2CD2:distal appendage proteins:TTBK2:MARK4ArrowR-HSA-5626699 (Reactome)
centrosome:C2CD2:distal appendage proteins:TTBK2:MARK4R-HSA-5626227 (Reactome)
dynein-2ArrowR-HSA-5625421 (Reactome)
dynein-2ArrowR-HSA-5625424 (Reactome)
dynein-2R-HSA-5624949 (Reactome)
exocyst complex:RAB8A:GTP:RAB3IP:RAB11:GTP:RAB11FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsArrowR-HSA-5623524 (Reactome)
exocyst complex:RAB8A:GTP:RAB3IP:RAB11:GTP:RAB11FIP3 dimer:ASAP1 dimer:VxPx-containing ciliary membrane proteinsR-HSA-5623527 (Reactome)
exocyst complexR-HSA-5623524 (Reactome)
mother centriole:C2CD3ArrowR-HSA-5626220 (Reactome)
mother centriole:C2CD3R-HSA-5626223 (Reactome)
mother centrioleR-HSA-5626220 (Reactome)
myristoylated ciliary cargoR-HSA-5624131 (Reactome)
myristoylated ciliary proteinsArrowR-HSA-5624130 (Reactome)
retrograde IFT trainsArrowR-HSA-5624952 (Reactome)
retrograde IFT trainsArrowR-HSA-5625426 (Reactome)
retrograde IFT trainsR-HSA-5625424 (Reactome)
retrograde IFT trainsR-HSA-5625426 (Reactome)
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