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
Peroxisome
hsa04146

All organismsOrtholog table
Disease
H00075  
Refsum disease
H00176  
Adrenoleukodystrophy (ALD)
H00177  
Adrenoleukodystrophy, neonatal (NALD)
H00203  
Acatalasia
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
D01206  
Febuxostat (JAN/USAN/INN)
D02365  
Oxypurinol (USAN)
D10386  
Cindunistat (USAN/INN)
D10419  
Cindunistat hydrochloride maleate (USAN)
Other DBs
BSID: 
GO: 
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/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, peroxisomal [KO:K13237] [EC:1.3.1.34]
1891  
ECH1; enoyl-CoA hydratase 1 [KO:K12663] [EC:5.3.3.-]
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 (exo-N4-amino) [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, soluble [KO:K04565] [EC:1.15.1.1]
6648  
SOD2; superoxide dismutase 2, mitochondrial [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)
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)
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)
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)
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
 

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