KEGG   PATHWAY: uar04146
Entry
uar04146                    Pathway                                
Name
Peroxisome - Ursus americanus (American black bear)
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
uar04146  Peroxisome
uar04146

Other DBs
GO: 0005777
Organism
Ursus americanus (American black bear) [GN:uar]
Gene
123799583  PEX16; peroxisomal biogenesis factor 16 [KO:K13335]
123800406  PEX3; peroxisomal biogenesis factor 3 [KO:K13336]
123783299  PEX19; peroxisomal biogenesis factor 19 [KO:K13337]
123795195  ABCD3; ATP-binding cassette sub-family D member 3 isoform X1 [KO:K05677] [EC:7.6.2.4]
123789292  PEX1; peroxisome biogenesis factor 1 isoform X1 [KO:K13338]
123794574  PEX6; peroxisome biogenesis factor 6 [KO:K13339]
123778149  PEX26; peroxisome assembly protein 26 isoform X1 [KO:K13340]
123803334  PEX7; peroxisomal biogenesis factor 7 [KO:K13341]
123786418  PEX5L; PEX5-related protein isoform X1 [KO:K13342]
123781621  PEX5; peroxisomal biogenesis factor 5 isoform X1 [KO:K13342]
123779110  PEX14; peroxisomal membrane protein PEX14 isoform X1 [KO:K13343]
123785959  PEX13; peroxisome biogenesis factor 13 [KO:K13344]
123777064  PEX12; peroxisome assembly protein 12 [KO:K13345]
123796126  PEX10; LOW QUALITY PROTEIN: peroxisome biogenesis factor 10 [KO:K13346]
123797949  PEX2; peroxisome biogenesis factor 2 [KO:K06664]
123803881  PXMP2; peroxisomal membrane protein 2 isoform X1 [KO:K13347]
123799910  MPV17; protein Mpv17 isoform X1 [KO:K13348]
123779940  MPV17L2; mpv17-like protein 2 [KO:K13348]
123778616  MPV17L; mpv17-like protein isoform X1 [KO:K13349]
123798304  PXMP4; peroxisomal membrane protein 4 isoform X1 [KO:K13350]
123802476  PEX11A; peroxisomal membrane protein 11A isoform X1 [KO:K13351]
123778686  PEX11B; peroxisomal membrane protein 11B [KO:K13352]
123777535  PEX11G; peroxisomal membrane protein 11C isoform X1 [KO:K13353]
123780646  SLC25A17; peroxisomal membrane protein PMP34 isoform X1 [KO:K13354]
123788357  HACL1; 2-hydroxyacyl-CoA lyase 1 isoform X1 [KO:K12261] [EC:4.1.2.63]
123785459  AMACR; alpha-methylacyl-CoA racemase isoform X1 [KO:K01796] [EC:5.1.99.4]
123786156  PHYH; phytanoyl-CoA dioxygenase, peroxisomal isoform X1 [KO:K00477] [EC:1.14.11.18]
123786332  phytanoyl-CoA dioxygenase, peroxisomal-like isoform X1 [KO:K00477] [EC:1.14.11.18]
123789992  phytanoyl-CoA dioxygenase, peroxisomal-like isoform X1 [KO:K00477] [EC:1.14.11.18]
123789993  phytanoyl-CoA dioxygenase, peroxisomal-like isoform X1 [KO:K00477] [EC:1.14.11.18]
123802961  ACOX1; peroxisomal acyl-coenzyme A oxidase 1 isoform X1 [KO:K00232] [EC:1.3.3.6]
123779190  ACOX3; peroxisomal acyl-coenzyme A oxidase 3 isoform X1 [KO:K00232] [EC:1.3.3.6]
123798800  ACOX2; peroxisomal acyl-coenzyme A oxidase 2 [KO:K10214] [EC:1.17.99.3]
123790430  HSD17B4; peroxisomal multifunctional enzyme type 2 [KO:K12405] [EC:1.1.1.- 4.2.1.107 4.2.1.119]
123793683  SCP2; sterol carrier protein 2 [KO:K08764] [EC:2.3.1.176]
123781224  acyl-coenzyme A amino acid N-acyltransferase 2-like [KO:K00659] [EC:2.3.1.65 3.1.2.2]
123781225  bile acid-CoA:amino acid N-acyltransferase-like [KO:K00659] [EC:2.3.1.65 3.1.2.2]
123778934  EHHADH; peroxisomal bifunctional enzyme [KO:K07514] [EC:4.2.1.17 1.1.1.35 5.3.3.8]
123792838  ACAA1; 3-ketoacyl-CoA thiolase, peroxisomal [KO:K07513] [EC:2.3.1.16]
123783323  DECR2; peroxisomal 2,4-dienoyl-CoA reductase [(3E)-enoyl-CoA-producing] [KO:K13237] [EC:1.3.1.124]
123784510  ECH1; delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, mitochondrial [KO:K12663] [EC:5.3.3.21]
123795578  ABCD1; ATP-binding cassette sub-family D member 1 [KO:K05675] [EC:7.6.2.4]
123778862  ABCD2; ATP-binding cassette sub-family D member 2 [KO:K05676] [EC:7.6.2.4]
123798989  ABCD4; lysosomal cobalamin transporter ABCD4 isoform X1 [KO:K05678] [EC:7.6.2.8]
123801850  SLC27A2; very long-chain acyl-CoA synthetase [KO:K08746] [EC:6.2.1.- 6.2.1.3]
123786628  ACSL4; long-chain-fatty-acid--CoA ligase 4 isoform X1 [KO:K01897] [EC:6.2.1.3]
123795963  ACSL5; long-chain-fatty-acid--CoA ligase 5 [KO:K01897] [EC:6.2.1.3]
123790376  ACSL6; long-chain-fatty-acid--CoA ligase 6 isoform X1 [KO:K01897] [EC:6.2.1.3]
123784509  ACSL1; long-chain-fatty-acid--CoA ligase 1 isoform X1 [KO:K01897] [EC:6.2.1.3]
123778088  ACSL3; fatty acid CoA ligase Acsl3 [KO:K01897] [EC:6.2.1.3]
123798281  PECR; peroxisomal trans-2-enoyl-CoA reductase [KO:K07753] [EC:1.3.1.38]
123801544  ECI2; enoyl-CoA delta isomerase 2 isoform X1 [KO:K13239] [EC:5.3.3.8]
123791327  NUDT7; peroxisomal coenzyme A diphosphatase NUDT7 isoform X1 [KO:K17879] [EC:3.6.1.77]
123799264  NUDT12; NAD-capped RNA hydrolase NUDT12 isoform X1 [KO:K03426] [EC:3.6.1.22]
123782971  NUDT13; NAD(P)H pyrophosphatase NUDT13, mitochondrial isoform X1 [KO:K03426] [EC:3.6.1.22]
123791385  NUDT19; acyl-coenzyme A diphosphatase NUDT19 [KO:K13355] [EC:3.6.1.77]
123797075  ACOT8; acyl-coenzyme A thioesterase 8 isoform X1 [KO:K11992] [EC:3.1.2.27]
123775617  CRAT; LOW QUALITY PROTEIN: carnitine O-acetyltransferase [KO:K00624] [EC:2.3.1.7]
123789333  CROT; peroxisomal carnitine O-octanoyltransferase isoform X1 [KO:K05940] [EC:2.3.1.137]
123791358  MLYCD; malonyl-CoA decarboxylase, mitochondrial [KO:K01578] [EC:4.1.1.9]
123782849  GNPAT; dihydroxyacetone phosphate acyltransferase isoform X1 [KO:K00649] [EC:2.3.1.42]
123798076  AGPS; alkyldihydroxyacetonephosphate synthase, peroxisomal isoform X1 [KO:K00803] [EC:2.5.1.26]
123801626  FAR2; fatty acyl-CoA reductase 2 [KO:K13356] [EC:1.2.1.84]
123791659  FAR1; LOW QUALITY PROTEIN: fatty acyl-CoA reductase 1 [KO:K13356] [EC:1.2.1.84]
123788211  MVK; mevalonate kinase [KO:K00869] [EC:2.7.1.36]
123783458  PMVK; phosphomevalonate kinase isoform X1 [KO:K13273] [EC:2.7.4.2]
123791242  AGXT; serine--pyruvate aminotransferase isoform X1 [KO:K00830] [EC:2.6.1.44 2.6.1.45 2.6.1.51]
123788223  DAO; D-amino-acid oxidase isoform X1 [KO:K00273] [EC:1.4.3.3]
123792950  DDO; D-aspartate oxidase isoform X1 [KO:K00272] [EC:1.4.3.1]
123802442  IDH2; isocitrate dehydrogenase [NADP], mitochondrial [KO:K00031] [EC:1.1.1.42]
123798256  IDH1; isocitrate dehydrogenase [NADP] cytoplasmic isoform X1 [KO:K00031] [EC:1.1.1.42]
123800009  PAOX; peroxisomal N(1)-acetyl-spermine/spermidine oxidase [KO:K00308] [EC:1.5.3.13]
123777004  PIPOX; peroxisomal sarcosine oxidase isoform X1 [KO:K00306] [EC:1.5.3.1 1.5.3.7]
123795778  HMGCLL1; 3-hydroxymethyl-3-methylglutaryl-CoA lyase, cytoplasmic [KO:K01640] [EC:4.1.3.4]
123782471  HMGCL; hydroxymethylglutaryl-CoA lyase, mitochondrial [KO:K01640] [EC:4.1.3.4]
123800575  HAO1; hydroxyacid oxidase 1 [KO:K11517] [EC:1.1.3.15]
123795009  HAO2; hydroxyacid oxidase 2 [KO:K11517] [EC:1.1.3.15]
123799428  CAT; catalase [KO:K03781] [EC:1.11.1.6]
123778325  PRDX5; peroxiredoxin-5, mitochondrial [KO:K11187] [EC:1.11.1.24]
123784194  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
123797500  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
123776978  NOS2; nitric oxide synthase, inducible isoform X1 [KO:K13241] [EC:1.14.13.39]
123793763  PRDX1; peroxiredoxin-1 [KO:K13279] [EC:1.11.1.24]
123777357  EPHX2; bifunctional epoxide hydrolase 2 isoform X1 [KO:K08726] [EC:3.3.2.10 3.1.3.76]
123788839  GSTK1; glutathione S-transferase kappa 1 [KO:K13299] [EC:2.5.1.18]
123797546  LOW QUALITY PROTEIN: glutathione S-transferase kappa 1-like [KO:K13299] [EC:2.5.1.18]
123799862  XDH; xanthine dehydrogenase/oxidase [KO:K00106] [EC:1.17.1.4 1.17.3.2]
123795764  dehydrogenase/reductase SDR family member 4 isoform X1 [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
uar00071  Fatty acid degradation
uar00120  Primary bile acid biosynthesis
uar00230  Purine metabolism
uar00564  Glycerophospholipid metabolism
uar00565  Ether lipid metabolism
uar00830  Retinol metabolism
uar00900  Terpenoid backbone biosynthesis
uar03320  PPAR signaling pathway
KO pathway
ko04146   
LinkDB

DBGET integrated database retrieval system