Peroxisomal lipid metabolism (Homo sapiens)

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118, 27, 326, 3419, 4411145, 39, 439, 1215421294, 10262, 362, 361, 2385, 39, 4320, 3525, 28, 315, 39, 4324, 413331511, 1630381, 17, 40216, 345, 39, 433813, 22, 25, 37GNPATAGPS complex HACL1 holoenzyme monomer IDH1 dimer AGPSFAD ABCD1 homodimer ACOX2FAD ACOX1FAD complex PHYHFe++ HACL1 tetramer cytosolIDH1 dimer peroxisomal matrixHSD17B4 dimer ACOX3FAD ACOX1 dimer 4,8-dimethylnonanoylcarnitineISCITGNPAT PPiNADP+H+FOR-CoACoA-SHCO2Malonyl-CoAHSD17B4 dimer3-ketopristanoyl-CoANAD+ATPNADHO2PHYH ACAA1CoA-SHACOX1-2 ACOX3 Octanoyl-CoAH+SLC25A17CoA-SHGO3PPropionylcarnitineNAD+trans-2,3-dehydropristanoyl-CoAAc-CoAAMACRFAD CoA-SHpropionyl CoAAADHAPRMLYCDFAD H+IDH1 NADP+2OGAMPIDH1 dimerPristanal dehydrogenase3-ketohexacosanoyl-CoAHCOOHMg2+ DHAPC260 CoACoA-SHABCD1 homodimerCoA-SHPalmCoAFAR2C260 CoAFAD H2OISCITGO3PCRATH+PALMPhytanoyl-CoACoA-SH3-hydroxyhexacosanoyl-CoAH2O2O2H2OCO2NAD+NADPHNAD+CARO24,8-dimethylnonanoyl-CoASLC27A2TPP PPiSCP2-1H2OABCD1 SUCCAAc-CoANADPHAc-CoAACOX2FADH+IDH1 Pristanic acidCROT4,8,12-trimethyltridecanoyl-CoANADP+ACARCH3COO-CARisocitrate-oxoglutarate transporterH+NAD+CoA-SHAGPS HSD17B4GNPATAGPS complexFe2+ H2O2AMPHXOLNADHATPAMPPHYHFe++trans-2,3-dehydrohexacosanoyl-CoA3S2HPhy-CoAO21-Palmitoyl dihydroxyacetone phosphateACOX1 dimerACOX2 NADH2OGH2OATPH+H2Otetracosanoyl-CoANADHHACL1 O2NADPHIDH1 dimerH2OPristanal3-hydroxypristanoyl-CoAPhytanateH2O2FAD ACOX3FADACOT8FAR1HACL1 tetramerHXDG3PH2O2AMP7, 3434


Description

In humans, the catabolism of phytanate, pristanate, and very long chain fatty acids as well as the first four steps of the biosynthesis of plasmalogens are catalyzed by peroxisomal enzymes. Defects in any of these enzymes or in the assembly of peroxisomes are associated with severe developmental disorders (Wanders and Watherham 2006). Original Pathway at Reactome: http://www.reactome.org/PathwayBrowser/#DB=gk_current&FOCUS_SPECIES_ID=48887&FOCUS_PATHWAY_ID=390918

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Bibliography

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  31. Croes K, Van Veldhoven PP, Mannaerts GP, Casteels M.; ''Production of formyl-CoA during peroxisomal alpha-oxidation of 3-methyl-branched fatty acids.''; PubMed Europe PMC Scholia
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  46. Vanhooren JC, Marynen P, Mannaerts GP, Van Veldhoven PP.; ''Evidence for the existence of a pristanoyl-CoA oxidase gene in man.''; PubMed Europe PMC Scholia
  47. Foulon V, Antonenkov VD, Croes K, Waelkens E, Mannaerts GP, Van Veldhoven PP, Casteels M.; ''Purification, molecular cloning, and expression of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal thiamine pyrophosphate-dependent enzyme that catalyzes the carbon-carbon bond cleavage during alpha-oxidation of 3-methyl-branched fatty acids.''; PubMed Europe PMC Scholia
  48. Gasmi L, McLennan AG.; ''The mouse Nudt7 gene encodes a peroxisomal nudix hydrolase specific for coenzyme A and its derivatives.''; PubMed Europe PMC Scholia
  49. Onwukwe GU, Kursula P, Koski MK, Schmitz W, Wierenga RK.; ''Human Δ³,Δ²-enoyl-CoA isomerase, type 2: a structural enzymology study on the catalytic role of its ACBP domain and helix-10.''; PubMed Europe PMC Scholia
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  51. Jiang LL, Kobayashi A, Matsuura H, Fukushima H, Hashimoto T.; ''Purification and properties of human D-3-hydroxyacyl-CoA dehydratase: medium-chain enoyl-CoA hydratase is D-3-hydroxyacyl-CoA dehydratase.''; PubMed Europe PMC Scholia
  52. Ferdinandusse S, Kostopoulos P, Denis S, Rusch H, Overmars H, Dillmann U, Reith W, Haas D, Wanders RJ, Duran M, Marziniak M.; ''Mutations in the gene encoding peroxisomal sterol carrier protein X (SCPx) cause leukencephalopathy with dystonia and motor neuropathy.''; PubMed Europe PMC Scholia
  53. Ferdinandusse S, Denis S, van Berkel E, Dacremont G, Wanders RJ.; ''Peroxisomal fatty acid oxidation disorders and 58 kDa sterol carrier protein X (SCPx). Activity measurements in liver and fibroblasts using a newly developed method.''; PubMed Europe PMC Scholia
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  55. Nazarko TY.; ''Atg37 regulates the assembly of the pexophagic receptor protein complex.''; PubMed Europe PMC Scholia

History

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CompareRevisionActionTimeUserComment
114994view16:52, 25 January 2021ReactomeTeamReactome version 75
113438view11:51, 2 November 2020ReactomeTeamReactome version 74
112641view16:02, 9 October 2020ReactomeTeamReactome version 73
101556view11:42, 1 November 2018ReactomeTeamreactome version 66
101092view21:25, 31 October 2018ReactomeTeamreactome version 65
100621view19:59, 31 October 2018ReactomeTeamreactome version 64
100172view16:44, 31 October 2018ReactomeTeamreactome version 63
99722view15:11, 31 October 2018ReactomeTeamreactome version 62 (2nd attempt)
99296view12:46, 31 October 2018ReactomeTeamreactome version 62
93948view13:47, 16 August 2017ReactomeTeamreactome version 61
93541view11:26, 9 August 2017ReactomeTeamreactome version 61
86640view09:22, 11 July 2016ReactomeTeamreactome version 56
83100view09:58, 18 November 2015ReactomeTeamVersion54
81430view12:57, 21 August 2015ReactomeTeamVersion53
76900view08:17, 17 July 2014ReactomeTeamFixed remaining interactions
76605view11:58, 16 July 2014ReactomeTeamFixed remaining interactions
75936view09:59, 11 June 2014ReactomeTeamRe-fixing comment source
75638view10:52, 10 June 2014ReactomeTeamReactome 48 Update
74993view13:51, 8 May 2014AnweshaFixing comment source for displaying WikiPathways description
74637view08:41, 30 April 2014ReactomeTeamReactome46
68924view17:32, 8 July 2013MaintBotUpdated to 2013 gpml schema
45246view18:32, 7 October 2011KhanspersOntology Term : 'lipid metabolic pathway' added !
42094view21:56, 4 March 2011MaintBotAutomatic update
39904view05:55, 21 January 2011MaintBotNew pathway

External references

DataNodes

View all...
NameTypeDatabase referenceComment
1-Palmitoyl dihydroxyacetone phosphateMetaboliteCHEBI:17868 (ChEBI)
2OGMetaboliteCHEBI:30915 (ChEBI)
3-hydroxyhexacosanoyl-CoAMetaboliteCHEBI:64972 (ChEBI)
3-hydroxypristanoyl-CoAMetaboliteCHEBI:63914 (ChEBI)
3-ketohexacosanoyl-CoAMetaboliteCHEBI:52977 (ChEBI)
3-ketopristanoyl-CoAMetaboliteCHEBI:15371 (ChEBI)
3S2HPhy-CoAMetaboliteCHEBI:15475 (ChEBI)
4,8,12-trimethyltridecanoyl-CoAMetaboliteCHEBI:15495 (ChEBI)
4,8-dimethylnonanoyl-CoAMetaboliteCHEBI:63856 (ChEBI)
4,8-dimethylnonanoylcarnitineMetaboliteCHEBI:63874 (ChEBI)
AADHAPRREACT_4622 (Reactome)
ABCD1 ProteinP33897 (Uniprot-TrEMBL)
ABCD1 homodimerComplexREACT_15606 (Reactome)
ACAA1ProteinP09110 (Uniprot-TrEMBL)
ACARMetaboliteCHEBI:15960 (ChEBI)
ACOT8ProteinO14734 (Uniprot-TrEMBL)
ACOX1 dimerComplexREACT_18252 (Reactome)
ACOX1-2 ProteinQ15067-2 (Uniprot-TrEMBL)
ACOX2 FADComplexREACT_10603 (Reactome)
ACOX2 ProteinQ99424 (Uniprot-TrEMBL)
ACOX3 FADComplexREACT_17430 (Reactome)
ACOX3 ProteinO15254 (Uniprot-TrEMBL)
AGPS ProteinO00116 (Uniprot-TrEMBL)
AMACRProteinQ9UHK6 (Uniprot-TrEMBL)
AMPMetaboliteCHEBI:16027 (ChEBI)
ATPMetaboliteCHEBI:15422 (ChEBI)
Ac-CoAMetaboliteCHEBI:15351 (ChEBI)
C26 0 CoAMetaboliteCHEBI:52966 (ChEBI)
CARMetaboliteCHEBI:17126 (ChEBI)
CH3COO-MetaboliteCHEBI:15366 (ChEBI)
MetaboliteCHEBI:51341 (ChEBI)
MetaboliteCHEBI:64039 (ChEBI)
CO2MetaboliteCHEBI:16526 (ChEBI)
CRATProteinP43155 (Uniprot-TrEMBL)
CROTProteinQ9UKG9 (Uniprot-TrEMBL)
CoA-SHMetaboliteCHEBI:15346 (ChEBI)
DHAPMetaboliteCHEBI:16108 (ChEBI)
FAD MetaboliteCHEBI:16238 (ChEBI)
FAR1ProteinQ8WVX9 (Uniprot-TrEMBL)
FAR2ProteinQ96K12 (Uniprot-TrEMBL)
FOR-CoAMetaboliteCHEBI:15522 (ChEBI)
Fe2+ MetaboliteCHEBI:18248 (ChEBI)
GNPAT AGPS complexComplexREACT_2259 (Reactome)
GNPAT ProteinO15228 (Uniprot-TrEMBL)
GO3PMetaboliteCHEBI:17197 (ChEBI)
H+MetaboliteCHEBI:15378 (ChEBI)
H2O2MetaboliteCHEBI:16240 (ChEBI)
H2OMetaboliteCHEBI:15377 (ChEBI)
HACL1 ProteinQ9UJ83 (Uniprot-TrEMBL)
HACL1 tetramerComplexREACT_17962 (Reactome)
HCOOHMetaboliteCHEBI:30751 (ChEBI)
HSD17B4 dimerComplexREACT_17840 (Reactome)
HSD17B4ProteinP51659 (Uniprot-TrEMBL)
HXDG3PMetaboliteCHEBI:63818 (ChEBI)
HXOLMetaboliteCHEBI:16125 (ChEBI)
IDH1 ProteinO75874 (Uniprot-TrEMBL)
IDH1 dimerComplexREACT_17197 (Reactome)
IDH1 dimerComplexREACT_17471 (Reactome)
ISCITMetaboliteCHEBI:151 (ChEBI)
MLYCDProteinO95822 (Uniprot-TrEMBL)
Malonyl-CoAMetaboliteCHEBI:15531 (ChEBI)
Mg2+ MetaboliteCHEBI:18420 (ChEBI)
NAD+MetaboliteCHEBI:15846 (ChEBI)
NADHMetaboliteCHEBI:16908 (ChEBI)
NADP+MetaboliteCHEBI:18009 (ChEBI)
NADPHMetaboliteCHEBI:16474 (ChEBI)
O2MetaboliteCHEBI:15379 (ChEBI)
Octanoyl-CoAMetaboliteCHEBI:15533 (ChEBI)
PALMMetaboliteCHEBI:15756 (ChEBI)
PHYH Fe++ComplexREACT_17895 (Reactome)
PHYH ProteinO14832 (Uniprot-TrEMBL)
PPiMetaboliteCHEBI:29888 (ChEBI)
PalmCoAMetaboliteCHEBI:15525 (ChEBI)
PhytanateMetaboliteCHEBI:16285 (ChEBI)
Phytanoyl-CoAMetaboliteCHEBI:15538 (ChEBI)
Pristanal dehydrogenaseREACT_17759 (Reactome)
PristanalMetaboliteCHEBI:49189 (ChEBI)
Pristanic acidMetaboliteCHEBI:51340 (ChEBI)
PropionylcarnitineMetaboliteCHEBI:28867 (ChEBI)
SCP2-1ProteinP22307-1 (Uniprot-TrEMBL)
SLC25A17ProteinO43808 (Uniprot-TrEMBL)
SLC27A2ProteinO14975 (Uniprot-TrEMBL)
SUCCAMetaboliteCHEBI:15741 (ChEBI)
TPP MetaboliteCHEBI:18290 (ChEBI)
isocitrate-oxoglutarate transporterREACT_18178 (Reactome)
propionyl CoAMetaboliteCHEBI:15539 (ChEBI)
tetracosanoyl-CoAMetaboliteCHEBI:52974 (ChEBI)
trans-2,3-dehydrohexacosanoyl-CoAMetaboliteCHEBI:52975 (ChEBI)
trans-2,3-dehydropristanoyl-CoAMetaboliteCHEBI:63803 (ChEBI)

Annotated Interactions

View all...
SourceTargetTypeDatabase referenceComment
1-Palmitoyl dihydroxyacetone phosphateArrowREACT_15 (Reactome)
1-Palmitoyl dihydroxyacetone phosphateREACT_16930 (Reactome)
2OGArrowREACT_16889 (Reactome)
2OGArrowREACT_16932 (Reactome)
2OGArrowREACT_17007 (Reactome)
2OGREACT_16889 (Reactome)
2OGREACT_17021 (Reactome)
3-hydroxyhexacosanoyl-CoAREACT_16971 (Reactome)
3-hydroxypristanoyl-CoAREACT_16976 (Reactome)
3-ketohexacosanoyl-CoAArrowREACT_16971 (Reactome)
3-ketohexacosanoyl-CoAREACT_16946 (Reactome)
3-ketopristanoyl-CoAArrowREACT_16976 (Reactome)
3-ketopristanoyl-CoAREACT_17019 (Reactome)
3S2HPhy-CoAArrowREACT_17021 (Reactome)
4,8,12-trimethyltridecanoyl-CoAArrowREACT_17019 (Reactome)
4,8,12-trimethyltridecanoyl-CoAREACT_16894 (Reactome)
4,8-dimethylnonanoyl-CoAArrowREACT_16894 (Reactome)
4,8-dimethylnonanoyl-CoAREACT_16985 (Reactome)
4,8-dimethylnonanoylcarnitineArrowREACT_16985 (Reactome)
AADHAPRREACT_759 (Reactome)
ABCD1 homodimerREACT_16917 (Reactome)
ACAA1REACT_16946 (Reactome)
ACARArrowREACT_16977 (Reactome)
ACOT8REACT_16974 (Reactome)
ACOX1 dimerREACT_17054 (Reactome)
ACOX2 FADREACT_17014 (Reactome)
ACOX3 FADREACT_16962 (Reactome)
AMACRREACT_17018 (Reactome)
AMPArrowREACT_16948 (Reactome)
AMPArrowREACT_17000 (Reactome)
AMPArrowREACT_17058 (Reactome)
AMPREACT_16948 (Reactome)
ATPArrowREACT_16948 (Reactome)
ATPREACT_16948 (Reactome)
ATPREACT_17000 (Reactome)
ATPREACT_17058 (Reactome)
Ac-CoAArrowREACT_163880 (Reactome)
Ac-CoAArrowREACT_16894 (Reactome)
Ac-CoAArrowREACT_16946 (Reactome)
Ac-CoAArrowREACT_17013 (Reactome)
Ac-CoAREACT_16974 (Reactome)
Ac-CoAREACT_16977 (Reactome)
ArrowREACT_17058 (Reactome)
C26 0 CoAREACT_17054 (Reactome)
CARREACT_16949 (Reactome)
CARREACT_16977 (Reactome)
CARREACT_16985 (Reactome)
CH3COO-ArrowREACT_16974 (Reactome)
CO2ArrowREACT_163880 (Reactome)
CO2ArrowREACT_16932 (Reactome)
CO2ArrowREACT_17007 (Reactome)
CO2ArrowREACT_17021 (Reactome)
CRATREACT_16949 (Reactome)
CRATREACT_16977 (Reactome)
CROTREACT_16985 (Reactome)
CoA-SHArrowREACT_15 (Reactome)
CoA-SHArrowREACT_16949 (Reactome)
CoA-SHArrowREACT_16974 (Reactome)
CoA-SHArrowREACT_16977 (Reactome)
CoA-SHArrowREACT_16985 (Reactome)
CoA-SHArrowREACT_16990 (Reactome)
CoA-SHArrowREACT_17006 (Reactome)
CoA-SHArrowREACT_17042 (Reactome)
CoA-SHREACT_16894 (Reactome)
CoA-SHREACT_16946 (Reactome)
CoA-SHREACT_17000 (Reactome)
CoA-SHREACT_17013 (Reactome)
CoA-SHREACT_17019 (Reactome)
CoA-SHREACT_17058 (Reactome)
DHAPREACT_15 (Reactome)
FAR1REACT_17006 (Reactome)
FAR2REACT_17042 (Reactome)
FOR-CoAArrowREACT_17035 (Reactome)
FOR-CoAREACT_16990 (Reactome)
GNPAT AGPS complexREACT_15 (Reactome)
GNPAT AGPS complexREACT_16930 (Reactome)
GO3PArrowREACT_16930 (Reactome)
GO3PREACT_759 (Reactome)
H+ArrowREACT_16894 (Reactome)
H+ArrowREACT_16919 (Reactome)
H+ArrowREACT_16932 (Reactome)
H+ArrowREACT_16971 (Reactome)
H+ArrowREACT_16976 (Reactome)
H+ArrowREACT_17007 (Reactome)
H+ArrowREACT_17013 (Reactome)
H+REACT_17006 (Reactome)
H+REACT_17042 (Reactome)
H+REACT_759 (Reactome)
H2O2ArrowREACT_16894 (Reactome)
H2O2ArrowREACT_16962 (Reactome)
H2O2ArrowREACT_17013 (Reactome)
H2O2ArrowREACT_17014 (Reactome)
H2O2ArrowREACT_17054 (Reactome)
H2OREACT_16894 (Reactome)
H2OREACT_16908 (Reactome)
H2OREACT_16944 (Reactome)
H2OREACT_16974 (Reactome)
H2OREACT_16990 (Reactome)
H2OREACT_17013 (Reactome)
HACL1 tetramerREACT_17035 (Reactome)
HCOOHArrowREACT_16990 (Reactome)
HSD17B4 dimerREACT_16908 (Reactome)
HSD17B4 dimerREACT_16944 (Reactome)
HSD17B4 dimerREACT_16971 (Reactome)
HSD17B4 dimerREACT_16976 (Reactome)
HXDG3PArrowREACT_759 (Reactome)
HXOLArrowREACT_17006 (Reactome)
HXOLArrowREACT_17042 (Reactome)
HXOLREACT_16930 (Reactome)
IDH1 dimerREACT_16932 (Reactome)
IDH1 dimerREACT_17007 (Reactome)
ISCITArrowREACT_16889 (Reactome)
ISCITREACT_16889 (Reactome)
ISCITREACT_16932 (Reactome)
ISCITREACT_17007 (Reactome)
MLYCDREACT_163880 (Reactome)
NAD+REACT_16894 (Reactome)
NAD+REACT_16919 (Reactome)
NAD+REACT_16971 (Reactome)
NAD+REACT_16976 (Reactome)
NAD+REACT_17013 (Reactome)
NADHArrowREACT_16894 (Reactome)
NADHArrowREACT_16919 (Reactome)
NADHArrowREACT_16971 (Reactome)
NADHArrowREACT_16976 (Reactome)
NADHArrowREACT_17013 (Reactome)
NADP+ArrowREACT_17006 (Reactome)
NADP+ArrowREACT_17042 (Reactome)
NADP+ArrowREACT_759 (Reactome)
NADP+REACT_16932 (Reactome)
NADP+REACT_17007 (Reactome)
NADPHArrowREACT_16932 (Reactome)
NADPHArrowREACT_17007 (Reactome)
NADPHREACT_17006 (Reactome)
NADPHREACT_17042 (Reactome)
NADPHREACT_759 (Reactome)
O2REACT_16894 (Reactome)
O2REACT_16962 (Reactome)
O2REACT_17013 (Reactome)
O2REACT_17014 (Reactome)
O2REACT_17021 (Reactome)
O2REACT_17054 (Reactome)
Octanoyl-CoAArrowREACT_17013 (Reactome)
PALMArrowREACT_16930 (Reactome)
PHYH Fe++REACT_17021 (Reactome)
PPiArrowREACT_17000 (Reactome)
PPiArrowREACT_17058 (Reactome)
PalmCoAREACT_15 (Reactome)
PalmCoAREACT_17006 (Reactome)
PalmCoAREACT_17042 (Reactome)
PhytanateREACT_17000 (Reactome)
Phytanoyl-CoAArrowREACT_17000 (Reactome)
Phytanoyl-CoAREACT_17021 (Reactome)
Pristanal dehydrogenaseREACT_16919 (Reactome)
PristanalArrowREACT_17035 (Reactome)
PristanalREACT_16919 (Reactome)
Pristanic acidArrowREACT_16919 (Reactome)
Pristanic acidREACT_17058 (Reactome)
PropionylcarnitineArrowREACT_16949 (Reactome)
REACT_15 (Reactome) Peroxisomal glyceronephosphate O-acyltransferase (GNPAT) catalyzes the reaction of palmitoyl-CoA and DHAP to form 1-palmitoyl glycerone phosphate (1-palmitoyl dihydroxyacetone phosphate) and CoASH. The active form of the enzyme is one subunit of a heterotrimer with two molecules of the alkylglycerone phosphate synthase (AGPS) enzyme (Biermann et al. 1999). It was first purified and characterized biochemically by Ofman and Wanders (1994). Mutations in the GNPAT gene are associated with rhizomelic chondrodysplasia type 2 (Ofman et al. 1998, 2001).
REACT_163880 (Reactome) Carboxylation of acetyl-CoA and decarboxylation of malonyl-CoA are two processes that can control the amount of the signal transducer malonyl-CoA in the cell. The decarboxylation is catalysed by MCD enzyme in the peroxisomal matrix (Sacksteder et al, 1999).
REACT_16889 (Reactome) A specific transport process that exchanges 2-oxoglutarate for isocitrate across a lipid membrane has been reconstituted in vitro with proteins purified from bovine peroxisomal membranes. The specific protein or proteins that mediate this transport process have not yet been identified in any mammalian system, however (Visser et al. 2006).
REACT_16894 (Reactome) In two cycles of beta-oxidation mediated by the same enzyme activities responsible for the conversion of pristanoyl-CoA to 4,8,12-trimethyltridecanoyl-CoA, the latter molecule is converted to 4,8-dimethylnonanoyl-CoA. Two molecules each of O2, H2O, NAD+, and CoASH are consumed in the process and two molecules of H2O2 and NADH + H+ are generated, together with single molecules of acetyl-CoA and propionyl-CoA (Verhoeven et al. 1998).
REACT_16908 (Reactome) Peroxisomal HSD17B4 dimer catalyzes the reaction of trans-2,3-dehydrohexacosanoyl-CoA and H2O to form 3-hydroxyhexacosanoyl-CoA. The enzyme is bifunctional - an aminoterminal domain catalyzes the dehydrogenation of a variety of 3-hydroxyacyl-CoA's and a carboxyterminal domain catalyzes the hydration of a variety of trans-2,3-dehydroacyl-CoA's, the reaction annotated here (Jiang et al. 1996, 1997). Defects in the enzyme are associated with a severe disorder of peroxisomal fatty acid metabolism in humans (Ferdinandusse et al. 2006).
REACT_16917 (Reactome) Homodimeric ABCD1 associated with the peroxisomal membrane mediates the uptake of cytosolic very long chain fatty acyl CoAs such as hexacosanoyl-CoA into the peroxisomal matrix. While the requirement for this uptake step in the catabolism of very long chain fatty acids is well-established, direct evidence for the function of ABCD1 as a transporter comes only from studies of its ability to restore peroxisomal long chain fatty acid catabolism in yeast strains whose endogenous transporters have been disrupted by mutation. ABCD1 is inferred to function as a dimer like other members of the ABCD transporter family. The energy requirements of peroxisomal fatty acid uptake (other ABCD transporter-mediated reactions are coupled to ATP hydrolysis) have not been established (van Roermund et al. 2008).
REACT_16919 (Reactome) Peroxisomes contain a pristanal dehydrogenase activity that catalyzes the reaction of pristanal and NAD+ to form pristanate and NADH + H+. The enzyme responsible for this activity has not yet been purified (Jansen et al. 2001).
REACT_16930 (Reactome) Peroxisomal alkylglycerone phosphate synthase (AGPS) catalyzes the reaction of 1-palmitoylglycerone phosphate and hexadecanol to form O-hexadecylglycerone phosphate and palmitate. The active form of the enzyme is post-translationally cleaved to remove its 58 aminoterminal residues, has a molecule of FAD as a cofactor, and occurs in the peroxisome as a complex with glyceronephosphate O-acyltransferase (GNPAT) (Biermann et al. 1999; Bierman and van den Bosch 1999; de Vet et al. 2000).
REACT_16932 (Reactome) Peroxisomal IDH1 (isocitrate dehydrogenase 1) homodimer catalyzes the reaction of isocitrate and NADP+ to form 2-oxoglutarate, CO2, and NADPH + H+. The same enzyme can also localize to the cytosol in at least some cell types (Geisbrecht and Gould 1999; Xu et al. 2004).
REACT_16944 (Reactome) Peroxisomal HSD17B4 dimer catalyzes the reaction of trans-2,3-dehydropristanoyl-CoA and H2O to form 3-hydroxypristanoyl-CoA. The enzyme is bifunctional - an aminoterminal domain catalyzes the dehydrogenation of a variety of 3-hydroxyacyl-CoA's and a carboxyterminal domain catalyzes the hydration of a variety of trans-2,3-dehydroacyl-CoA's, the reaction annotated here (Jiang et al. 1996, 1997). Defects in the enzyme are associated with a severe disorder of peroxisomal fatty acid metabolism in humans (Ferdinandusse et al. 2006).
REACT_16946 (Reactome) Peroxisomal ACAA1 catalyzes the reaction of 3-ketohexacosanoyl-CoA and CoASH to form tetracosanoyl-CoA + acetyl-CoA (Bout et al. 1991).
REACT_16948 (Reactome) The peroxisomal membrane transport protein PMP34 mediates the exchange of adenine nucleotides between the cytosol and the peroxisomal matrix. The localization of PMP34 has been established by immunofluoresence studies (Wylin et al. 1998). The cloned human protein restores adenine nucleotide transport in yeast whose endogenous peroxisomal transporter has been disrupted, and has adenine nucleotide transport activity in reconstituted lipid vesicles in vitro (Visser et al. 2002), consistent with its hypothesized role in vivo (Wanders and Waterham 2006).
REACT_16949 (Reactome) Peroxisomal carnitineacetyltransferase (CRAT) catalyzes the reaction of propionyl-CoA and carnitine to form propionylcarnitine and CoASH. The active form of the enzyme is a monomer (Bloisi et al. 1990; Wu et al. 2003).
REACT_16962 (Reactome)
REACT_16971 (Reactome) Peroxisomal HSD17B4 dimer catalyzes the reaction of 3-hydroxyhexacosanoyl-CoA and NAD+ to form 3-ketohexacosanoyl-CoA and NADH + H+. The enzyme is bifunctional - an aminoterminal domain catalyzes the dehydrogenation of a variety of 3-hydroxyacyl-CoA's, the reaction annotated here, and a carboxyterminal domain catalyzes the hydration of a variety of trans-2,3-dehydroacyl-CoA's (Jiang et al. 1996, 1997). Defects in the enzyme are associated with a severe disorder of peroxisomal fatty acid metabolism in humans (Ferdinandusse et al. 2006).
REACT_16974 (Reactome) Peroxisomal ACOT8 catalyzes the hydrolysis of acetyl-CoA to form acetate and CoASH (Jones et al. 1999; Wanders and Waterham 2006).
REACT_16976 (Reactome) Peroxisomal HSD17B4 dimer catalyzes the reaction of 3-hydroxypristanoyl-CoA and NAD+ to form 3-ketoxypristanoyl-CoA and NADH + H+. The enzyme is bifunctional - an aminoterminal domain catalyzes the dehydrogenation of a variety of 3-hydroxyacyl-CoA's, the reaction annotated here, and a carboxyterminal domain catalyzes the hydration of a variety of trans-2,3-dehydroacyl-CoA's (Jiang et al. 1996, 1997). Defects in the enzyme are associated with a severe disorder of peroxisomal fatty acid metabolism in humans (Ferdinandusse et al. 2006).
REACT_16977 (Reactome) Peroxisomal carnitineacetyltransferase (CRAT) catalyzes the reaction of acetyl-CoA and carnitine to form acetylcarnitine and CoASH. The active form of the enzyme is a monomer (Bloisi et al. 1990; Wu et al. 2003).
REACT_16985 (Reactome) Peroxisomal CROT catalyzes the reaction of 4,8-dimethylnonanoyl-CoA and carnitine to form 4,8-dimethylnonanoylcarnitine and CoASH (Ferdinandusse et al. 1999).
REACT_16990 (Reactome) Formyl-CoA formed during the alpha-oxidation of phytanoyl-CoA is spontaneously hydrolyzed to formate and CoASH (Croes et al. 1997).
REACT_16999 (Reactome) O-hexadecylglycerone phosphate is synthesized in the peroxisomal matrix but its further metabolism takes place in the cytosol and endoplasmic reticulum. The mechanism by which it leaves the peroxisome is unknown (Nagan and Zoeller 2001).
REACT_17000 (Reactome) VLCS (very long chain acyl-CoA synthetase), associated with the inner surface of the peroxisomal membrane, cayalyzes the reaction of phytanate, CoA-SH, and ATP to form phytanoyl-CoA, AMP, and pyrophosphate (Steinberg et al. 1999).
REACT_17006 (Reactome) Peroxisomal fatty acyl-CoA reductase 1 (FAR1) catalyzes the reaction of palmitoyl-CoA and 2 NADPH + 2 H+ to form hexadecanol, CoASH, and 2 NADP+. As judged from mRNA levels, FAR1 is widely expressed in the body (Cheng and Russell 2004).
REACT_17007 (Reactome) Cytosolic IDH1 (isocitrate dehydrogenase 1) homodimer catalyzes the reaction of isocitrate and NADP+ to form 2-oxoglutarate, CO2, and NADPH + H+. The same enzyme can also localize to peroxisomes (Geisbrecht and Gould 1999; Xu et al. 2004).
REACT_17013 (Reactome) In eight cycles of beta-oxidation mediated by the same enzyme activities responsible for the conversion of hexacosanoyl-CoA to tetracosenoyl-CoA, the latter molecule is converted to octanoyl-CoA. Eight molecules each of O2, H2O, NAD+, and CoASH are consumed in the process and eight molecules of H2O2 and NADH + H+ are generated, together with eight molecules of acetyl-CoA (Wanders and Waterham 2006).
REACT_17014 (Reactome)
REACT_17018 (Reactome) Peroxisomal 2-methylacyl-CoA racemase (AMACR) catalyzes the isomerization of (2R)-pristanoyl-CoA to form (2S)-pristanoyl-CoA. The active form of the enzyme is a monomer (Schmitz et al. 1995; Amery et al. 2000; Ferdinandusse et al. 2000).
REACT_17019 (Reactome) Peroxisomal SCPx (Non-specific lipid transfer protein; SCP2) catalyzes the reaction of 3-ketopristanoyl-CoA and CoASH to form 4,8,12-trimethyltridecanoyl-CoA and propionyl-CoA. Both intact SCPx and an SCPx fragment corresponding to approximately the 430 aminoterminal residues of the protein are catalytically active in vitro; the latter form may predominate in vivo. Consistent with the role of SCPx in the beta-oxidation of branched-chain fatty acids in vitro, mutations in the protein are associated with elevated levels of pristanic acid in the blood in vivo and the development of neurological defects (Ferdinandusse et al. 2000, 2006).
REACT_17021 (Reactome) Peroxisomal phytanoyl-CoA dioxygenase catalyzes the reaction of phytanoyl-CoA, 2-oxoglutarate, and O2 to form 2-hydroxyphytanoyl-CoA, succinate, and CO2. The mature form of the enzyme lacks the first 30 amino acid residues of the full-length polypeptide and is complexed with Fe++. Mutations in this enzyme are the commonest cause of Refsum disease (Mukherji et al. 2001; McDonough et al. 2005).
REACT_17035 (Reactome) Peroxisomal HACL1 catalyzes the reaction of 2-hydroxyphytanoyl-CoA to form pristanal and formyl-CoA. The active form of the enzyme is a homotetramer, with one Mg++ and one molecule of thiamin pyrophosphate bound to each monomer (Croes et al. 1997; Foulon et al. 1999).
REACT_17042 (Reactome) Peroxisomal fatty acyl-CoA reductase 2 (FAR2) catalyzes the reaction of palmitoyl-CoA and 2 NADPH + 2 H+ to form hexadecanol, CoASH, and 2 NADP+. As judged from mRNA levels, FAR2 is not widely expressed in the body but is abundant in brain (Cheng and Russell 2004).
REACT_17054 (Reactome) Peroxisomal ACOX1 catalyzes the reaction of hexacosanoyl-CoA and O2 to form trans-2,3-dehydrohexacosanoyl-CoA and H2O2. The active form of the enzyme is a homodimer with FAD as a cofactor (Chu et al. 1995). Mutations in the ACOX1 gene are asociated with accumulation of very long chain fatty acids. Two isoforms of ACOX1, generated by alternative splicing are known; a mutation affecting specifically the second isoform blocks the oxidation of very long chain fatty acids (Ferdinandusse et al. 2007).
REACT_17058 (Reactome) VLCS (very long chain acyl-CoA synthetase), associated with the inner surface of the peroxisomal membrane, catalyzes the reaction of pristanate, CoA-SH and ATP to form pristanoyl-CoA, AMP and pyrophosphate (Steinberg et al. 1999).
REACT_759 (Reactome) AADHAPR catalyzes the reaction of O-hexadecyldihydroxyacetone phosphate and NADPH + H+ to form 1-hexadecyl glycerol-3-phosphate and NADP+. The enzyme is associated with the outer face of the peroxisomal membrane; its substrates and products are thought to be cytosolic. The enzyme appears to be well-conserved among animal species. The properties of the human enzyme are inferred from those of an activity partially purified from guinea pig liver (Datta et al. 1990).
SCP2-1REACT_17019 (Reactome)
SLC25A17REACT_16948 (Reactome)
SLC27A2REACT_17000 (Reactome)
SLC27A2REACT_17058 (Reactome)
SUCCAArrowREACT_17021 (Reactome)
isocitrate-oxoglutarate transporterREACT_16889 (Reactome)
propionyl CoAArrowREACT_16894 (Reactome)
propionyl CoAArrowREACT_17019 (Reactome)
propionyl CoAREACT_16949 (Reactome)
tetracosanoyl-CoAArrowREACT_16946 (Reactome)
tetracosanoyl-CoAREACT_17013 (Reactome)
trans-2,3-dehydrohexacosanoyl-CoAArrowREACT_17054 (Reactome)
trans-2,3-dehydrohexacosanoyl-CoAREACT_16908 (Reactome)
trans-2,3-dehydropristanoyl-CoAArrowREACT_16962 (Reactome)
trans-2,3-dehydropristanoyl-CoAArrowREACT_17014 (Reactome)
trans-2,3-dehydropristanoyl-CoAREACT_16944 (Reactome)
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