KEGG   PATHWAY: eee04146
Entry
eee04146                    Pathway                                
Name
Peroxisome - Electrophorus electricus (electric eel)
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
Pathway map
eee04146  Peroxisome
eee04146

Other DBs
GO: 0005777
Organism
Electrophorus electricus (electric eel) [GN:eee]
Gene
113579054  pex16; peroxisomal biogenesis factor 16 [KO:K13335]
113583162  pex3; peroxisomal biogenesis factor 3 [KO:K13336]
113580212  pex19; peroxisomal biogenesis factor 19 isoform X1 [KO:K13337]
113590951  ATP-binding cassette sub-family D member 3-like isoform X1 [KO:K05677] [EC:7.6.2.4]
113580751  abcd3; ATP-binding cassette sub-family D member 3 isoform X1 [KO:K05677] [EC:7.6.2.4]
113578679  pex1; peroxisome biogenesis factor 1 isoform X1 [KO:K13338]
113568896  pex6; peroxisome biogenesis factor 6 [KO:K13339]
113578098  pex26; peroxisome assembly protein 26 [KO:K13340]
113570805  pex7; peroxisomal biogenesis factor 7 [KO:K13341]
113579526  pex5l; PEX5-related protein isoform X1 [KO:K13342]
113591746  PEX5-related protein-like isoform X1 [KO:K13342]
113570619  pex5; peroxisomal biogenesis factor 5 isoform X1 [KO:K13342]
113581300  pex14; peroxisomal membrane protein PEX14 [KO:K13343]
113582181  pex13; peroxisome biogenesis factor 13 [KO:K13344]
113573711  pex12; peroxisome assembly protein 12 isoform X1 [KO:K13345]
113578200  pex10; peroxisome biogenesis factor 10 [KO:K13346]
113573887  pex2; peroxisome biogenesis factor 2 [KO:K06664]
113588112  pxmp2; peroxisomal membrane protein 2 [KO:K13347]
113571590  mpv17; protein Mpv17 isoform X1 [KO:K13348]
113581899  mpv17l2; mpv17-like protein 2 [KO:K13348]
113580877  mpv17l; mpv17-like protein [KO:K13349]
113575260  mpv17-like protein isoform X1 [KO:K13349]
113569175  pxmp4; peroxisomal membrane protein 4 [KO:K13350]
113573984  pex11a; peroxisomal membrane protein 11A [KO:K13351]
113590446  pex11b; peroxisomal membrane protein 11B [KO:K13352]
113569876  peroxisomal membrane protein PMP34-like isoform X1 [KO:K13354]
113571243  hacl1; 2-hydroxyacyl-CoA lyase 1 [KO:K12261] [EC:4.1.2.63]
113570768  amacr; alpha-methylacyl-CoA racemase isoform X1 [KO:K01796] [EC:5.1.99.4]
113591541  phyh; phytanoyl-CoA dioxygenase, peroxisomal [KO:K00477] [EC:1.14.11.18]
113583258  acox1; peroxisomal acyl-coenzyme A oxidase 1 isoform X1 [KO:K00232] [EC:1.3.3.6]
113573498  acox3; LOW QUALITY PROTEIN: peroxisomal acyl-coenzyme A oxidase 3 [KO:K00232] [EC:1.3.3.6]
113571049  hsd17b4; peroxisomal multifunctional enzyme type 2 [KO:K12405] [EC:1.1.1.- 4.2.1.107 4.2.1.119]
113570128  scp2; non-specific lipid-transfer protein [KO:K08764] [EC:2.3.1.176]
113584003  acyl-coenzyme A thioesterase 1-like [KO:K00659] [EC:2.3.1.65 3.1.2.2]
113574639  ehhadh; peroxisomal bifunctional enzyme isoform X1 [KO:K07514] [EC:4.2.1.17 1.1.1.35 5.3.3.8]
113581209  acaa1; 3-ketoacyl-CoA thiolase, peroxisomal [KO:K07513] [EC:2.3.1.16]
113586569  decr2; peroxisomal 2,4-dienoyl-CoA reductase isoform X1 [KO:K13237] [EC:1.3.1.124]
113584659  ech1; delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, mitochondrial isoform X1 [KO:K12663] [EC:5.3.3.21]
113575837  abcd1; ATP-binding cassette sub-family D member 1 [KO:K05675] [EC:7.6.2.4]
113580180  ATP-binding cassette sub-family D member 2-like [KO:K05676] [EC:7.6.2.4]
113581652  abcd4; ATP-binding cassette sub-family D member 4 isoform X1 [KO:K05678] [EC:7.6.2.8]
113581763  very long-chain acyl-CoA synthetase-like [KO:K08746] [EC:6.2.1.- 6.2.1.3]
113585358  very long-chain acyl-CoA synthetase-like isoform X1 [KO:K08746] [EC:6.2.1.- 6.2.1.3]
113577296  very long-chain acyl-CoA synthetase-like [KO:K08746] [EC:6.2.1.- 6.2.1.3]
113578022  acsl6; long-chain-fatty-acid--CoA ligase 6 isoform X1 [KO:K01897] [EC:6.2.1.3]
113581601  acsl4; long-chain-fatty-acid--CoA ligase 4 [KO:K01897] [EC:6.2.1.3]
113581733  long-chain-fatty-acid--CoA ligase 1-like [KO:K01897] [EC:6.2.1.3]
113587539  acsl5; long-chain-fatty-acid--CoA ligase 5 [KO:K01897] [EC:6.2.1.3]
113592057  LOW QUALITY PROTEIN: long-chain-fatty-acid--CoA ligase 1-like [KO:K01897] [EC:6.2.1.3]
113592269  long-chain-fatty-acid--CoA ligase 4-like [KO:K01897] [EC:6.2.1.3]
113578146  long-chain-fatty-acid--CoA ligase 1-like isoform X1 [KO:K01897] [EC:6.2.1.3]
113590461  LOW QUALITY PROTEIN: long-chain-fatty-acid--CoA ligase 3-like [KO:K01897] [EC:6.2.1.3]
113587009  long-chain-fatty-acid--CoA ligase 3-like [KO:K01897] [EC:6.2.1.3]
113577716  peroxisomal trans-2-enoyl-CoA reductase-like [KO:K07753] [EC:1.3.1.38]
113579389  eci2; enoyl-CoA delta isomerase 2, mitochondrial isoform X1 [KO:K13239] [EC:5.3.3.8]
113585314  nudt7; peroxisomal coenzyme A diphosphatase NUDT7 isoform X1 [KO:K17879] [EC:3.6.1.77]
113571066  nudt12; peroxisomal NADH pyrophosphatase NUDT12 isoform X1 [KO:K03426] [EC:3.6.1.22]
113587486  nudt13; nucleoside diphosphate-linked moiety X motif 13 isoform X1 [KO:K03426] [EC:3.6.1.22]
113576710  nudt19; nucleoside diphosphate-linked moiety X motif 19 [KO:K13355] [EC:3.6.1.77]
113572378  acot8; acyl-coenzyme A thioesterase 8 [KO:K11992] [EC:3.1.2.27]
113578197  carnitine O-acetyltransferase-like isoform X1 [KO:K00624] [EC:2.3.1.7]
113589074  carnitine O-acetyltransferase-like isoform X1 [KO:K00624] [EC:2.3.1.7]
113570225  crat; carnitine O-acetyltransferase [KO:K00624] [EC:2.3.1.7]
113577078  crot; peroxisomal carnitine O-octanoyltransferase [KO:K05940] [EC:2.3.1.137]
113568093  mlycd; malonyl-CoA decarboxylase, mitochondrial [KO:K01578] [EC:4.1.1.9]
113577045  gnpat; dihydroxyacetone phosphate acyltransferase [KO:K00649] [EC:2.3.1.42]
113568113  dihydroxyacetone phosphate acyltransferase-like isoform X1 [KO:K00649] [EC:2.3.1.42]
113582330  agps; alkyldihydroxyacetonephosphate synthase, peroxisomal isoform X1 [KO:K00803] [EC:2.5.1.26]
113576554  fatty acyl-CoA reductase 1-like [KO:K13356] [EC:1.2.1.84]
113582491  fatty acyl-CoA reductase 1-like [KO:K13356] [EC:1.2.1.84]
113592108  mevalonate kinase-like [KO:K00869] [EC:2.7.1.36]
113584898  pmvk; phosphomevalonate kinase isoform X1 [KO:K13273] [EC:2.7.4.2]
113575923  serine--pyruvate aminotransferase, mitochondrial-like isoform X1 [KO:K00830] [EC:2.6.1.44 2.6.1.45 2.6.1.51]
113587628  serine--pyruvate aminotransferase-like [KO:K00830] [EC:2.6.1.44 2.6.1.45 2.6.1.51]
113576941  dao; D-amino-acid oxidase [KO:K00273] [EC:1.4.3.3]
113578157  ddo; D-aspartate oxidase [KO:K00272] [EC:1.4.3.1]
113573975  idh2; isocitrate dehydrogenase [NADP], mitochondrial [KO:K00031] [EC:1.1.1.42]
113567506  idh1; isocitrate dehydrogenase [NADP] cytoplasmic [KO:K00031] [EC:1.1.1.42]
113580961  paox; peroxisomal N(1)-acetyl-spermine/spermidine oxidase isoform X1 [KO:K00308] [EC:1.5.3.13]
113569729  pipox; peroxisomal sarcosine oxidase isoform X1 [KO:K00306] [EC:1.5.3.1 1.5.3.7]
113588062  hmgcl; hydroxymethylglutaryl-CoA lyase, mitochondrial isoform X1 [KO:K01640] [EC:4.1.3.4]
113585405  hmgcll1; 3-hydroxymethyl-3-methylglutaryl-CoA lyase, cytoplasmic isoform X1 [KO:K01640] [EC:4.1.3.4]
113587745  hao2; hydroxyacid oxidase 2 [KO:K11517] [EC:1.1.3.15]
113572674  hao1; hydroxyacid oxidase 1 isoform X1 [KO:K11517] [EC:1.1.3.15]
113573651  cat; catalase [KO:K03781] [EC:1.11.1.6]
113590815  prdx5; LOW QUALITY PROTEIN: peroxiredoxin-5, mitochondrial [KO:K11187] [EC:1.11.1.24]
113571779  sod1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
113570553  sod2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
113591429  nitric oxide synthase, inducible-like [KO:K13241] [EC:1.14.13.39]
113570157  peroxiredoxin-1 [KO:K13279] [EC:1.11.1.24]
113572683  ephx2; bifunctional epoxide hydrolase 2 [KO:K08726] [EC:3.3.2.10 3.1.3.76]
113573080  gstk1; glutathione S-transferase kappa 1 isoform X1 [KO:K13299] [EC:2.5.1.18]
113583665  xdh; xanthine dehydrogenase/oxidase [KO:K00106] [EC:1.17.1.4 1.17.3.2]
113578295  dehydrogenase/reductase SDR family member 4-like [KO:K11147] [EC:1.1.-.-]
Reference
PMID:17055078
  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
PMID:17055079
  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
PMID:17010456
  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
PMID:16631601
  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
Related
pathway
eee00071  Fatty acid degradation
eee00120  Primary bile acid biosynthesis
eee00230  Purine metabolism
eee00564  Glycerophospholipid metabolism
eee00565  Ether lipid metabolism
eee00830  Retinol metabolism
eee00900  Terpenoid backbone biosynthesis
eee03320  PPAR signaling pathway
KO pathway
ko04146   
LinkDB

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