KEGG   PATHWAY: xtr04146
Entry
xtr04146                    Pathway                                
Name
Peroxisome - Xenopus tropicalis (tropical clawed frog)
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
xtr04146  Peroxisome
xtr04146

Other DBs
GO: 0005777
Organism
Xenopus tropicalis (tropical clawed frog) [GN:xtr]
Gene
100145130  pex16; peroxisomal membrane protein PEX16 [KO:K13335]
733783  pex3; peroxisomal biogenesis factor 3 [KO:K13336]
100145099  pex19; peroxisomal biogenesis factor 19 [KO:K13337]
100489671  abcd3; ATP-binding cassette sub-family D member 3 [KO:K05677] [EC:7.6.2.4]
779516  pex1; peroxisome biogenesis factor 1 isoform X2 [KO:K13338]
100491422  pex6; peroxisome biogenesis factor 6 [KO:K13339]
100145166  pex26; peroxisome assembly protein 26 [KO:K13340]
548708  pex7; peroxisomal biogenesis factor 7 [KO:K13341]
496849  pex5; peroxisomal biogenesis factor 5 [KO:K13342]
100498559  pex5l; PEX5-related protein [KO:K13342]
100489925  pex14; peroxisomal membrane protein PEX14 [KO:K13343]
733832  pex13; peroxisome biogenesis factor 13 [KO:K13344]
100380099  pex12; peroxisome assembly protein 12 [KO:K13345]
100487506  pex10; peroxisome biogenesis factor 10 [KO:K13346]
549595  pex2; peroxisome biogenesis factor 2 [KO:K06664]
448721  pxmp2; peroxisomal membrane protein 2 [KO:K13347]
100125162  mpv17; uncharacterized protein LOC100125162 [KO:K13348]
448319  mpv17l2; mpv17-like protein 2 [KO:K13348]
100497159  mpv17-like protein 2 [KO:K13348]
100493944  mpv17l; mpv17-like protein [KO:K13349]
100380078  pxmp4; peroxisomal membrane protein 4 [KO:K13350]
595093  pex11a; peroxisomal membrane protein 11A [KO:K13351]
100379866  pex11b; peroxisomal membrane protein 11B [KO:K13352]
780227  pex11g; peroxisomal membrane protein 11C [KO:K13353]
448001  slc25a17; peroxisomal membrane protein PMP34 [KO:K13354]
550086  hacl1; 2-hydroxyacyl-CoA lyase 1 [KO:K12261] [EC:4.1.2.63]
493271  amacr; alpha-methylacyl-CoA racemase [KO:K01796] [EC:5.1.99.4]
100379998  phyh; phytanoyl-CoA dioxygenase, peroxisomal [KO:K00477] [EC:1.14.11.18]
548479  acox1; peroxisomal acyl-coenzyme A oxidase 1 [KO:K00232] [EC:1.3.3.6]
100380064  acox3; peroxisomal acyl-coenzyme A oxidase 3 [KO:K00232] [EC:1.3.3.6]
548740  acox2; peroxisomal acyl-coenzyme A oxidase 2 [KO:K10214] [EC:1.17.99.3]
613082  hsd17b4; peroxisomal multifunctional enzyme type 2 [KO:K12405] [EC:1.1.1.- 4.2.1.107 4.2.1.119]
548512  scp2; non-specific lipid-transfer protein [KO:K08764] [EC:2.3.1.176]
100125079  baat; bile acid-CoA:amino acid N-acyltransferase [KO:K00659] [EC:2.3.1.65 3.1.2.2]
100497580  acyl-coenzyme A amino acid N-acyltransferase 1 isoform X2 [KO:K00659] [EC:2.3.1.65 3.1.2.2]
100496940  acyl-coenzyme A amino acid N-acyltransferase 2 [KO:K00659] [EC:2.3.1.65 3.1.2.2]
100497260  LOW QUALITY PROTEIN: acyl-coenzyme A amino acid N-acyltransferase 2 [KO:K00659] [EC:2.3.1.65 3.1.2.2]
100497423  acyl-coenzyme A amino acid N-acyltransferase 2 [KO:K00659] [EC:2.3.1.65 3.1.2.2]
100497738  acyl-coenzyme A amino acid N-acyltransferase 2 [KO:K00659] [EC:2.3.1.65 3.1.2.2]
100488333  acyl-coenzyme A amino acid N-acyltransferase 2-like [KO:K00659] [EC:2.3.1.65 3.1.2.2]
116408197  acyl-coenzyme A amino acid N-acyltransferase 2-like [KO:K00659] [EC:2.3.1.65 3.1.2.2]
100216115  ehhadh; peroxisomal bifunctional enzyme [KO:K07514] [EC:4.2.1.17 1.1.1.35 5.3.3.8]
780143  acaa1; 3-ketoacyl-CoA thiolase, peroxisomal [KO:K07513] [EC:2.3.1.16]
394884  decr2; peroxisomal 2,4-dienoyl-CoA reductase isoform X1 [KO:K13237] [EC:1.3.1.124]
448218  ech1; delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, mitochondrial [KO:K12663] [EC:5.3.3.21]
100493545  abcd1; ATP-binding cassette sub-family D member 1 [KO:K05675] [EC:7.6.2.4]
101730598  abcd2; ATP-binding cassette sub-family D member 2 [KO:K05676] [EC:7.6.2.4]
100493343  abcd4; ATP-binding cassette sub-family D member 4 isoform X1 [KO:K05678] [EC:7.6.2.8]
100494612  slc27a3; very long-chain acyl-CoA synthetase [KO:K08746] [EC:6.2.1.- 6.2.1.3]
613067  slc27a2; very long-chain acyl-CoA synthetase [KO:K08746] [EC:6.2.1.- 6.2.1.3]
448565  acsl1; long-chain-fatty-acid--CoA ligase 1 [KO:K01897] [EC:6.2.1.3]
496479  acsl5; long-chain-fatty-acid--CoA ligase 5 [KO:K01897] [EC:6.2.1.3]
779783  acsl6; long-chain-fatty-acid--CoA ligase 6 [KO:K01897] [EC:6.2.1.3]
100036652  acsl3; long-chain-fatty-acid--CoA ligase 3 [KO:K01897] [EC:6.2.1.3]
100494759  acsl4; long-chain-fatty-acid--CoA ligase 4 isoform X2 [KO:K01897] [EC:6.2.1.3]
496922  pecr; peroxisomal trans-2-enoyl-CoA reductase [KO:K07753] [EC:1.3.1.38]
100216270  eci2; enoyl-CoA delta isomerase 2, mitochondrial [KO:K13239] [EC:5.3.3.8]
100490656  eci3; enoyl-CoA delta isomerase 2, mitochondrial [KO:K13239] [EC:5.3.3.8]
100379717  nudt7; peroxisomal coenzyme A diphosphatase NUDT7 [KO:K17879] [EC:3.6.1.77]
594920  nudt12; peroxisomal NADH pyrophosphatase NUDT12 [KO:K03426] [EC:3.6.1.22]
100492118  nudt13; nucleoside diphosphate-linked moiety X motif 13 [KO:K03426] [EC:3.6.1.22]
448257  nudt19; nucleoside diphosphate-linked moiety X motif 19 [KO:K13355] [EC:3.6.1.77]
100495154  acot8; acyl-coenzyme A thioesterase 8 [KO:K11992] [EC:3.1.2.27]
394811  crat; carnitine O-acetyltransferase b [KO:K00624] [EC:2.3.1.7]
613081  crata; carnitine O-acetyltransferase [KO:K00624] [EC:2.3.1.7]
733879  crot; peroxisomal carnitine O-octanoyltransferase [KO:K05940] [EC:2.3.1.137]
100145491  mlycd; malonyl-CoA decarboxylase, mitochondrial [KO:K01578] [EC:4.1.1.9]
733943  gnpat; dihydroxyacetone phosphate acyltransferase [KO:K00649] [EC:2.3.1.42]
100494829  dihydroxyacetone phosphate acyltransferase [KO:K00649] [EC:2.3.1.42]
100145360  agps; alkyldihydroxyacetonephosphate synthase, peroxisomal [KO:K00803] [EC:2.5.1.26]
100038270  far1; fatty acyl-CoA reductase 1 [KO:K13356] [EC:1.2.1.84]
100492928  mvk; mevalonate kinase isoform X1 [KO:K00869] [EC:2.7.1.36]
116406889  pmvk; phosphomevalonate kinase isoform X1 [KO:K13273] [EC:2.7.4.2]
448338  agxt; serine--pyruvate aminotransferase [KO:K00830] [EC:2.6.1.44 2.6.1.45 2.6.1.51]
100036595  dao; D-amino-acid oxidase [KO:K00273] [EC:1.4.3.3]
100488513  ddo; D-aspartate oxidase isoform X2 [KO:K00272] [EC:1.4.3.1]
448026  idh2; isocitrate dehydrogenase [NADP], mitochondrial [KO:K00031] [EC:1.1.1.42]
448164  idh1; isocitrate dehydrogenase [NADP] cytoplasmic [KO:K00031] [EC:1.1.1.42]
496840  paox; peroxisomal N(1)-acetyl-spermine/spermidine oxidase [KO:K00308] [EC:1.5.3.13]
100145496  uncharacterized protein LOC100145496 [KO:K00308] [EC:1.5.3.13]
548382  pipox; peroxisomal sarcosine oxidase [KO:K00306] [EC:1.5.3.1 1.5.3.7]
100494904  hmgcl; hydroxymethylglutaryl-CoA lyase, mitochondrial isoform X2 [KO:K01640] [EC:4.1.3.4]
100497263  hmgcll1; 3-hydroxymethyl-3-methylglutaryl-CoA lyase, cytoplasmic [KO:K01640] [EC:4.1.3.4]
595012  hao2; hydroxyacid oxidase 2 [KO:K11517] [EC:1.1.3.15]
100145574  hao1; hydroxyacid oxidase 1 [KO:K11517] [EC:1.1.3.15]
496897  cat.3; catalase, gene 3 [KO:K03781] [EC:1.11.1.6]
549470  cat.1; catalase, gene 1 [KO:K03781] [EC:1.11.1.6]
548403  cat.2; catalase, gene 2 [KO:K03781] [EC:1.11.1.6]
100494804  catalase [KO:K03781] [EC:1.11.1.6]
100127718  prdx5; peroxiredoxin-5, mitochondrial [KO:K11187] [EC:1.11.1.24]
549006  sod1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
448200  sod2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
100494698  nos2; nitric oxide synthase, inducible [KO:K13241] [EC:1.14.13.39]
496551  prdx1; peroxiredoxin-1 [KO:K13279] [EC:1.11.1.24]
448759  ephx2; bifunctional epoxide hydrolase 2 [KO:K08726] [EC:3.3.2.10 3.1.3.76]
496688  gstk1; glutathione S-transferase kappa 1 [KO:K13299] [EC:2.5.1.18]
100497499  xdh; xanthine dehydrogenase/oxidase [KO:K00106] [EC:1.17.1.4 1.17.3.2]
548501  dhrs4; dehydrogenase/reductase SDR family member 4 [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
xtr00071  Fatty acid degradation
xtr00120  Primary bile acid biosynthesis
xtr00230  Purine metabolism
xtr00564  Glycerophospholipid metabolism
xtr00565  Ether lipid metabolism
xtr00830  Retinol metabolism
xtr00900  Terpenoid backbone biosynthesis
xtr03320  PPAR signaling pathway
KO pathway
ko04146   
LinkDB

DBGET integrated database retrieval system