Peroxisomal lipid metabolism (Homo sapiens)

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224219, 3810, 16, 3721, 29333630, 3324, 323619, 382214396, 11, 225, 26, 31278, 92718, 23110, 16, 3721, 29320, 25, 31, 412, 174, 2210, 16, 37287, 124310, 16, 374013, 15, 35cytosolperoxisomal matrixtetracosanoyl-CoAACOT8ACOX3:FADAcetyl-CoA3-hydroxyhexacosanoyl-CoAPHYH:Fe++CoA-SHO-hexadecylglycerone phosphateO-hexadecylglycerone phosphateCRATPMP34H+CoA-SHisocitrate-oxoglutarate transporterFAR2Acetylcarnitine(2R) Pristanoyl-CoAH2O2NAD+palmitoyl-CoAABCD1 homodimerCoA-SHCarnitineDihydroxyacetone phosphatePyrophosphateNADP+NADPH2-oxoglutarateH+ACOX2:FADHSD17B4 dimerPristanal dehydrogenase3-ketopristanoyl-CoACoA-SHPropionylcarnitineCoA-SHNAD+Hexacosanoyl-CoAAMPH2O21-Palmitoyl dihydroxyacetone phosphateH2OOxygenHACL1 tetramerATPOctanoyl-CoAFormate(2S) Pristanoyl-CoAPristanalIDH1 dimerHexadecanolPyrophosphateHexacosanoyl-CoA3-hydroxypristanoyl-CoAIDH1 dimerOxygenNAD+NADHtrans-2,3-dehydrohexacosanoyl-CoAtrans-2,3-dehydropristanoyl-CoAOxygenAMACRAADHAPRpropionyl CoAFAR1NADPHNAD+4,8-dimethylnonanoylcarnitineACAA1Pristanic acidCoA-SH1-hexadecylglycerol-3-phosphateH2OH+NADHAMPH2OCarnitineAcetyl-CoAPalmitateNADHNADP+SCP2ATPIsocitrateCROTH+H2O24,8,12-trimethyltridecanoyl-CoASuccinateH2OH+2-OxoglutaratePhytanateNAD+2-Hydroxyphytanoyl-CoASLC27A2CoA-SHIsocitrateH+CO2NADPHOxygenATPH+ACOX1 dimerPhytanoyl-CoANADHH2O2H2OAMPFormyl-CoACoA-SHH2OGNPAT:AGPS complexAMP3-ketohexacosanoyl-CoANADP+OxygenCO24,8-dimethylnonanoyl-CoAAcetate29, 3429


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).

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Bibliography

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  7. Wylin T, Baes M, Brees C, Mannaerts GP, Fransen M, Van Veldhoven PP.; ''Identification and characterization of human PMP34, a protein closely related to the peroxisomal integral membrane protein PMP47 of Candida boidinii.''; PubMed Europe PMC Scholia
  8. Jones JM, Nau K, Geraghty MT, Erdmann R, Gould SJ.; ''Identification of peroxisomal acyl-CoA thioesterases in yeast and humans.''; PubMed Europe PMC Scholia
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  21. De Nys K, Meyhi E, Mannaerts GP, Fransen M, Van Veldhoven PP.; ''Characterisation of human peroxisomal 2,4-dienoyl-CoA reductase.''; PubMed Europe PMC Scholia
  22. Ferdinandusse S, Mulders J, IJlst L, Denis S, Dacremont G, Waterham HR, Wanders RJ.; ''Molecular cloning and expression of human carnitine octanoyltransferase: evidence for its role in the peroxisomal beta-oxidation of branched-chain fatty acids.''; PubMed Europe PMC Scholia
  23. McDonough MA, Kavanagh KL, Butler D, Searls T, Oppermann U, Schofield CJ.; ''Structure of human phytanoyl-CoA 2-hydroxylase identifies molecular mechanisms of Refsum disease.''; PubMed Europe PMC Scholia
  24. Ashibe B, Hirai T, Higashi K, Sekimizu K, Motojima K.; ''Dual subcellular localization in the endoplasmic reticulum and peroxisomes and a vital role in protecting against oxidative stress of fatty aldehyde dehydrogenase are achieved by alternative splicing.''; PubMed Europe PMC Scholia
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  26. Geisbrecht BV, Zhang D, Schulz H, Gould SJ.; ''Characterization of PECI, a novel monofunctional Delta(3), Delta(2)-enoyl-CoA isomerase of mammalian peroxisomes.''; PubMed Europe PMC Scholia
  27. Jones JM, Morrell JC, Gould SJ.; ''Identification and characterization of HAOX1, HAOX2, and HAOX3, three human peroxisomal 2-hydroxy acid oxidases.''; PubMed Europe PMC Scholia
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  30. Hunt MC, Solaas K, Kase BF, Alexson SE.; ''Characterization of an acyl-coA thioesterase that functions as a major regulator of peroxisomal lipid metabolism.''; PubMed Europe PMC Scholia
  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
  32. Jansen GA, van den Brink DM, Ofman R, Draghici O, Dacremont G, Wanders RJ.; ''Identification of pristanal dehydrogenase activity in peroxisomes: conclusive evidence that the complete phytanic acid alpha-oxidation pathway is localized in peroxisomes.''; PubMed Europe PMC Scholia
  33. Bout A, Franse MM, Collins J, Blonden L, Tager JM, Benne R.; ''Characterization of the gene encoding human peroxisomal 3-oxoacyl-CoA thiolase (ACAA). No large DNA rearrangement in a thiolase-deficient patient.''; PubMed Europe PMC Scholia
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  35. Rizzo WB, Lin Z, Carney G.; ''Fatty aldehyde dehydrogenase: genomic structure, expression and mutation analysis in Sjögren-Larsson syndrome.''; PubMed Europe PMC Scholia
  36. Ofman R, Speijer D, Leen R, Wanders RJ.; ''Proteomic analysis of mouse kidney peroxisomes: identification of RP2p as a peroxisomal nudix hydrolase with acyl-CoA diphosphatase activity.''; PubMed Europe PMC Scholia
  37. Nazarko TY, Ozeki K, Till A, Ramakrishnan G, Lotfi P, Yan M, Subramani S.; ''Peroxisomal Atg37 binds Atg30 or palmitoyl-CoA to regulate phagophore formation during pexophagy.''; PubMed Europe PMC Scholia
  38. Hunt MC, Rautanen A, Westin MA, Svensson LT, Alexson SE.; ''Analysis of the mouse and human acyl-CoA thioesterase (ACOT) gene clusters shows that convergent, functional evolution results in a reduced number of human peroxisomal ACOTs.''; PubMed Europe PMC Scholia
  39. Vanhove GF, Van Veldhoven PP, Fransen M, Denis S, Eyssen HJ, Wanders RJ, Mannaerts GP.; ''The CoA esters of 2-methyl-branched chain fatty acids and of the bile acid intermediates di- and trihydroxycoprostanic acids are oxidized by one single peroxisomal branched chain acyl-CoA oxidase in human liver and kidney.''; PubMed Europe PMC Scholia
  40. Kelson TL, Secor McVoy JR, Rizzo WB.; ''Human liver fatty aldehyde dehydrogenase: microsomal localization, purification, and biochemical characterization.''; PubMed Europe PMC Scholia
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  42. Das AK, Uhler MD, Hajra AK.; ''Molecular cloning and expression of mammalian peroxisomal trans-2-enoyl-coenzyme A reductase cDNAs.''; PubMed Europe PMC Scholia
  43. Ferdinandusse S, Ylianttila MS, Gloerich J, Koski MK, Oostheim W, Waterham HR, Hiltunen JK, Wanders RJ, Glumoff T.; ''Mutational spectrum of D-bifunctional protein deficiency and structure-based genotype-phenotype analysis.''; PubMed Europe PMC Scholia
  44. Lalwani ND, Reddy MK, Mangkornkanok-Mark M, Reddy JK.; ''Induction, immunochemical identity and immunofluorescence localization of an 80 000-molecular-weight peroxisome-proliferation-associated polypeptide (polypeptide PPA-80) and peroxisomal enoyl-CoA hydratase of mouse liver and renal cortex.''; PubMed Europe PMC Scholia
  45. Hua T, Wu D, Ding W, Wang J, Shaw N, Liu ZJ.; ''Studies of human 2,4-dienoyl CoA reductase shed new light on peroxisomal β-oxidation of unsaturated fatty acids.''; PubMed Europe PMC Scholia
  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
  50. Gloerich J, Ruiter JP, van den Brink DM, Ofman R, Ferdinandusse S, Wanders RJ.; ''Peroxisomal trans-2-enoyl-CoA reductase is involved in phytol degradation.''; PubMed Europe PMC Scholia
  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
  54. Wu D, Govindasamy L, Lian W, Gu Y, Kukar T, Agbandje-McKenna M, McKenna R.; ''Structure of human carnitine acetyltransferase. Molecular basis for fatty acyl transfer.''; PubMed Europe PMC Scholia
  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
(2R) Pristanoyl-

CoA

Metabolite51341 (ChEBI)
(2S) Pristanoyl-

CoA

MetaboliteREACT_17371 (Reactome)
1-Palmitoyl

dihydroxyacetone phosphate

Metabolite17868 (ChEBI)
1-hexadecylglycerol-

3-phosphate

MetaboliteREACT_18220 (Reactome)
2-

Hydroxyphytanoyl- CoA

Metabolite15475 (ChEBI)
2-Oxoglutarate Metabolite30915 (ChEBI)
2-oxoglutarate Metabolite30915 (ChEBI)
3-

hydroxyhexacosanoyl- CoA

MetaboliteREACT_17869 (Reactome)
3-

hydroxypristanoyl -CoA

MetaboliteREACT_17101 (Reactome)
3-

ketopristanoyl- CoA

MetaboliteREACT_18106 (Reactome)
3-ketohexacosanoyl-

CoA

MetaboliteREACT_17480 (Reactome)
4,8,12-

trimethyltridecanoyl -CoA

MetaboliteREACT_18090 (Reactome)
4,8-

dimethylnonanoyl carnitine

MetaboliteREACT_17091 (Reactome)
4,8-dimethylnonanoyl-

CoA

MetaboliteREACT_18121 (Reactome)
AADHAPR REACT_4622 (Reactome)
ABCD1

homodimer

ComplexREACT_15606 (Reactome)
ACAA1 ProteinP09110 (UniProt)
ACOT8 ProteinO14734 (UniProt)
ACOX1

dimer

ComplexREACT_18252 (Reactome)
ACOX2:

FAD

ComplexREACT_10603 (Reactome)
ACOX3:

FAD

ComplexREACT_17430 (Reactome)
AMACR ProteinQ9UHK6 (UniProt)
AMP Metabolite16027 (ChEBI)
ATP Metabolite15422 (ChEBI)
Acetate Metabolite15366 (ChEBI)
Acetyl-CoA Metabolite15351 (ChEBI)
Acetylcarnitine Metabolite15960 (ChEBI)
CO2 Metabolite16526 (ChEBI)
CRAT ProteinP43155 (UniProt)
CROT ProteinQ9UKG9 (UniProt)
Carnitine Metabolite17126 (ChEBI)
CoA-SH Metabolite15346 (ChEBI)
Dihydroxyacetone

phosphate

Metabolite16108 (ChEBI)
FAR1 ProteinQ8WVX9 (UniProt)
FAR2 ProteinQ96K12 (UniProt)
Formate Metabolite30751 (ChEBI)
Formyl-CoA Metabolite15522 (ChEBI)
GNPAT:AGPS

complex

ComplexREACT_2259 (Reactome)
H+ Metabolite15378 (ChEBI)
H2O Metabolite15377 (ChEBI)
H2O2 Metabolite16240 (ChEBI)
HACL1

tetramer

ComplexREACT_17962 (Reactome)
HSD17B4

dimer

ComplexREACT_17840 (Reactome)
Hexacosanoyl-

CoA

MetaboliteREACT_17364 (Reactome)
Hexacosanoyl-

CoA

MetaboliteREACT_17913 (Reactome)
Hexadecanol Metabolite16125 (ChEBI)
IDH1

dimer

ComplexREACT_17197 (Reactome)
IDH1

dimer

ComplexREACT_17471 (Reactome)
Isocitrate Metabolite151 (ChEBI)
NAD+ Metabolite15846 (ChEBI)
NADH Metabolite16908 (ChEBI)
NADP+ Metabolite18009 (ChEBI)
NADPH Metabolite16474 (ChEBI)
O-hexadecylglycerone

phosphate

Metabolite17197 (ChEBI)
Octanoyl-CoA Metabolite15533 (ChEBI)
Oxygen Metabolite15379 (ChEBI)
PHYH:

Fe++

ComplexREACT_17895 (Reactome)
PMP34 ProteinO43808 (UniProt)
Palmitate Metabolite15756 (ChEBI)
Phytanate Metabolite16285 (ChEBI)
Phytanoyl-CoA Metabolite15538 (ChEBI)
Pristanal Metabolite49189 (ChEBI)
Pristanal

dehydrogenase

REACT_17759 (Reactome)
Pristanic acid Metabolite51340 (ChEBI)
Propionylcarnitine Metabolite28867 (ChEBI)
Pyrophosp

hate

Metabolite29888 (ChEBI)
SCP2 ProteinP22307-1 (UniProt)
SLC27A2 ProteinO14975 (UniProt)
Succinate Metabolite15741 (ChEBI)
isocitrate-

oxoglutarate transporter

REACT_18178 (Reactome)
palmitoyl-

CoA

Metabolite15525 (ChEBI)
propionyl

CoA

Metabolite15539 (ChEBI)
tetracosanoyl-CoA MetaboliteREACT_17646 (Reactome)
trans-2,3-

dehydrohexacosanoyl- CoA

MetaboliteREACT_18221 (Reactome)
trans-2,3-

dehydropristanoyl- CoA

MetaboliteREACT_17125 (Reactome)

Annotated Interactions

No annotated interactions

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