KEGG   PATHWAY: hsa04146Help
Entry
hsa04146                    Pathway                                

Name
Peroxisome - Homo sapiens (human)
Description
Peroxisomes are essential organelles that play a key role in redox signalling and lipid homeostasis. They contribute to many crucial metabolic processes such as fatty acid oxidation, biosynthesis of ether lipids and free radical detoxification. The biogenesis of peroxisomes starts with the early peroxins PEX3, PEX16 and PEX19 and proceeds via several steps. The import of membrane proteins into peroxisomes needs PEX19 for recognition, targeting and insertion via docking at PEX3. Matrix proteins in the cytosol are recognized by peroxisomal targeting signals (PTS) and transported to the docking complex at the peroxisomal membrane. Peroxisomes' deficiencies lead to severe and often fatal inherited peroxisomal disorders (PD). PDs are usually classified in two groups. The first group is disorders of peroxisome biogenesis which include Zellweger syndrome, and the second group is single peroxisomal enzyme deficiencies.
Class
Cellular Processes; Transport and catabolism
BRITE hierarchy
Pathway map
hsa04146  Peroxisome
hsa04146

Ortholog table
Disease
H00075  Refsum disease
H00176  Adrenoleukodystrophy (ALD)
H00177  Adrenoleukodystrophy, neonatal (NALD)
H00203  Acatalasemia
H00205  Peroxisome biogenesis disorder
H00207  Rhizomelic chondrodysplasia punctata
H00407  Peroxisomal beta-oxidation enzyme deficiency
H00628  Congenital bile acid synthesis defect (CBAS)
H00874  Leukoencephalopathy with dystonia and motor neuropathy
H01155  Roussy-Levy syndrome
H01283  Malonyl-CoA decarboxylase deficiency
H01342  Zellweger syndrome (ZS)
Drug
D02365  Oxypurinol (USAN)
D10386  Cindunistat (USAN/INN)
D10419  Cindunistat hydrochloride maleate (USAN)
D10901  Enasidenib (USAN/INN)
D11044  Enasidenib mesylate (USAN)
Other DBs
BSID: 131226
GO: 0005777
Organism
Homo sapiens (human) [GN:hsa]
Gene
9409  PEX16; peroxisomal biogenesis factor 16 [KO:K13335]
8504  PEX3; peroxisomal biogenesis factor 3 [KO:K13336]
5824  PEX19; peroxisomal biogenesis factor 19 [KO:K13337]
5825  ABCD3; ATP binding cassette subfamily D member 3 [KO:K05677]
5189  PEX1; peroxisomal biogenesis factor 1 [KO:K13338]
5190  PEX6; peroxisomal biogenesis factor 6 [KO:K13339]
55670  PEX26; peroxisomal biogenesis factor 26 [KO:K13340]
5191  PEX7; peroxisomal biogenesis factor 7 [KO:K13341]
5830  PEX5; peroxisomal biogenesis factor 5 [KO:K13342]
51555  PEX5L; peroxisomal biogenesis factor 5 like [KO:K13342]
5195  PEX14; peroxisomal biogenesis factor 14 [KO:K13343]
5194  PEX13; peroxisomal biogenesis factor 13 [KO:K13344]
5193  PEX12; peroxisomal biogenesis factor 12 [KO:K13345]
5192  PEX10; peroxisomal biogenesis factor 10 [KO:K13346]
5828  PEX2; peroxisomal biogenesis factor 2 [KO:K06664]
5827  PXMP2; peroxisomal membrane protein 2 [KO:K13347]
4358  MPV17; MPV17, mitochondrial inner membrane protein [KO:K13348]
84769  MPV17L2; MPV17 mitochondrial inner membrane protein like 2 [KO:K13348]
255027  MPV17L; MPV17 mitochondrial inner membrane protein like [KO:K13349]
11264  PXMP4; peroxisomal membrane protein 4 [KO:K13350]
8800  PEX11A; peroxisomal biogenesis factor 11 alpha [KO:K13351]
8799  PEX11B; peroxisomal biogenesis factor 11 beta [KO:K13352]
92960  PEX11G; peroxisomal biogenesis factor 11 gamma [KO:K13353]
10478  SLC25A17; solute carrier family 25 member 17 [KO:K13354]
26061  HACL1; 2-hydroxyacyl-CoA lyase 1 [KO:K12261] [EC:4.1.-.-]
23600  AMACR; alpha-methylacyl-CoA racemase [KO:K01796] [EC:5.1.99.4]
5264  PHYH; phytanoyl-CoA 2-hydroxylase [KO:K00477] [EC:1.14.11.18]
8310  ACOX3; acyl-CoA oxidase 3, pristanoyl [KO:K00232] [EC:1.3.3.6]
51  ACOX1; acyl-CoA oxidase 1 [KO:K00232] [EC:1.3.3.6]
8309  ACOX2; acyl-CoA oxidase 2 [KO:K10214] [EC:1.17.99.3]
3295  HSD17B4; hydroxysteroid 17-beta dehydrogenase 4 [KO:K12405] [EC:4.2.1.119 4.2.1.107 1.1.1.35]
6342  SCP2; sterol carrier protein 2 [KO:K08764] [EC:2.3.1.176]
570  BAAT; bile acid-CoA:amino acid N-acyltransferase [KO:K00659] [EC:3.1.2.2 2.3.1.65]
1962  EHHADH; enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase [KO:K07514] [EC:5.3.3.8 1.1.1.35 4.2.1.17]
30  ACAA1; acetyl-CoA acyltransferase 1 [KO:K07513] [EC:2.3.1.16]
26063  DECR2; 2,4-dienoyl-CoA reductase 2 [KO:K13237] [EC:1.3.1.34]
1891  ECH1; enoyl-CoA hydratase 1 [KO:K12663] [EC:5.3.3.21]
215  ABCD1; ATP binding cassette subfamily D member 1 [KO:K05675]
225  ABCD2; ATP binding cassette subfamily D member 2 [KO:K05676]
5826  ABCD4; ATP binding cassette subfamily D member 4 [KO:K05678]
11001  SLC27A2; solute carrier family 27 member 2 [KO:K08746] [EC:6.2.1.3 6.2.1.-]
23305  ACSL6; acyl-CoA synthetase long chain family member 6 [KO:K01897] [EC:6.2.1.3]
2182  ACSL4; acyl-CoA synthetase long chain family member 4 [KO:K01897] [EC:6.2.1.3]
2180  ACSL1; acyl-CoA synthetase long chain family member 1 [KO:K01897] [EC:6.2.1.3]
51703  ACSL5; acyl-CoA synthetase long chain family member 5 [KO:K01897] [EC:6.2.1.3]
2181  ACSL3; acyl-CoA synthetase long chain family member 3 [KO:K01897] [EC:6.2.1.3]
55825  PECR; peroxisomal trans-2-enoyl-CoA reductase [KO:K07753] [EC:1.3.1.38]
10455  ECI2; enoyl-CoA delta isomerase 2 [KO:K13239] [EC:5.3.3.8]
283927  NUDT7; nudix hydrolase 7 [KO:K17879] [EC:3.6.1.-]
83594  NUDT12; nudix hydrolase 12 [KO:K03426] [EC:3.6.1.22]
390916  NUDT19; nudix hydrolase 19 [KO:K13355] [EC:3.6.1.-]
10005  ACOT8; acyl-CoA thioesterase 8 [KO:K11992] [EC:3.1.2.27]
1384  CRAT; carnitine O-acetyltransferase [KO:K00624] [EC:2.3.1.7]
54677  CROT; carnitine O-octanoyltransferase [KO:K05940] [EC:2.3.1.137]
23417  MLYCD; malonyl-CoA decarboxylase [KO:K01578] [EC:4.1.1.9]
8443  GNPAT; glyceronephosphate O-acyltransferase [KO:K00649] [EC:2.3.1.42]
8540  AGPS; alkylglycerone phosphate synthase [KO:K00803] [EC:2.5.1.26]
55711  FAR2; fatty acyl-CoA reductase 2 [KO:K13356] [EC:1.2.1.84]
84188  FAR1; fatty acyl-CoA reductase 1 [KO:K13356] [EC:1.2.1.84]
4598  MVK; mevalonate kinase [KO:K00869] [EC:2.7.1.36]
10654  PMVK; phosphomevalonate kinase [KO:K13273] [EC:2.7.4.2]
189  AGXT; alanine-glyoxylate aminotransferase [KO:K00830] [EC:2.6.1.51 2.6.1.45 2.6.1.44]
1610  DAO; D-amino acid oxidase [KO:K00273] [EC:1.4.3.3]
8528  DDO; D-aspartate oxidase [KO:K00272] [EC:1.4.3.1]
3417  IDH1; isocitrate dehydrogenase (NADP(+)) 1, cytosolic [KO:K00031] [EC:1.1.1.42]
3418  IDH2; isocitrate dehydrogenase (NADP(+)) 2, mitochondrial [KO:K00031] [EC:1.1.1.42]
196743  PAOX; polyamine oxidase [KO:K00308] [EC:1.5.3.13]
51268  PIPOX; pipecolic acid and sarcosine oxidase [KO:K00306] [EC:1.5.3.7 1.5.3.1]
3155  HMGCL; 3-hydroxymethyl-3-methylglutaryl-CoA lyase [KO:K01640] [EC:4.1.3.4]
54511  HMGCLL1; 3-hydroxymethyl-3-methylglutaryl-CoA lyase like 1 [KO:K01640] [EC:4.1.3.4]
51179  HAO2; hydroxyacid oxidase 2 [KO:K11517] [EC:1.1.3.15]
54363  HAO1; hydroxyacid oxidase 1 [KO:K11517] [EC:1.1.3.15]
847  CAT; catalase [KO:K03781] [EC:1.11.1.6]
25824  PRDX5; peroxiredoxin 5 [KO:K11187] [EC:1.11.1.15]
6647  SOD1; superoxide dismutase 1 [KO:K04565] [EC:1.15.1.1]
6648  SOD2; superoxide dismutase 2 [KO:K04564] [EC:1.15.1.1]
4843  NOS2; nitric oxide synthase 2 [KO:K13241] [EC:1.14.13.39]
5052  PRDX1; peroxiredoxin 1 [KO:K13279] [EC:1.11.1.15]
2053  EPHX2; epoxide hydrolase 2 [KO:K08726] [EC:3.1.3.76 3.3.2.10]
373156  GSTK1; glutathione S-transferase kappa 1 [KO:K13299] [EC:2.5.1.18]
7498  XDH; xanthine dehydrogenase [KO:K00106] [EC:1.17.3.2 1.17.1.4]
10901  DHRS4; dehydrogenase/reductase 4 [KO:K11147] [EC:1.1.-.-]
728635  DHRS4L1; dehydrogenase/reductase 4 like 1 [KO:K11147] [EC:1.1.-.-]
Reference
  Authors
Wanders RJ, Waterham HR
  Title
Peroxisomal disorders: the single peroxisomal enzyme deficiencies.
  Journal
Biochim Biophys Acta 1763:1707-20 (2006)
DOI:10.1016/j.bbamcr.2006.08.010
Reference
  Authors
Steinberg SJ, Dodt G, Raymond GV, Braverman NE, Moser AB, Moser HW
  Title
Peroxisome biogenesis disorders.
  Journal
Biochim Biophys Acta 1763:1733-48 (2006)
DOI:10.1016/j.bbamcr.2006.09.010
Reference
  Authors
Rottensteiner H, Theodoulou FL
  Title
The ins and outs of peroxisomes: co-ordination of membrane transport and peroxisomal metabolism.
  Journal
Biochim Biophys Acta 1763:1527-40 (2006)
DOI:10.1016/j.bbamcr.2006.08.012
Reference
  Authors
Iida R, Yasuda T, Tsubota E, Takatsuka H, Matsuki T, Kishi K
  Title
Human Mpv17-like protein is localized in peroxisomes and regulates expression of antioxidant enzymes.
  Journal
Biochem Biophys Res Commun 344:948-54 (2006)
DOI:10.1016/j.bbrc.2006.04.008
Reference
  Authors
Kleinman R, Goulet OJ, Mieli-Vergani G, Sanderson I, Sherman P, Shneider B.
  Title
Walker's Pediatric Gastrointestinal Disease (5th edition)
  Journal
B.C.Decker (2008)
KO pathway
ko04146   

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