KEGG   PATHWAY: cfr04146
Entry
cfr04146                    Pathway                                
Name
Peroxisome - Camelus ferus (Wild Bactrian camel)
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
cfr04146  Peroxisome
cfr04146

Other DBs
GO: 0005777
Organism
Camelus ferus (Wild Bactrian camel) [GN:cfr]
Gene
102506392  PEX16; peroxisomal biogenesis factor 16 [KO:K13335]
102507272  PEX3; peroxisomal biogenesis factor 3 isoform X1 [KO:K13336]
102517654  PEX19; LOW QUALITY PROTEIN: peroxisomal biogenesis factor 19 [KO:K13337]
102511665  ABCD3; ATP-binding cassette sub-family D member 3 isoform X3 [KO:K05677] [EC:7.6.2.4]
102519961  PEX1; LOW QUALITY PROTEIN: peroxisome biogenesis factor 1 [KO:K13338]
102507997  PEX6; peroxisome biogenesis factor 6 [KO:K13339]
102521870  PEX26; peroxisome assembly protein 26 [KO:K13340]
102514657  PEX7; peroxisomal biogenesis factor 7 [KO:K13341]
102515105  PEX5; peroxisomal biogenesis factor 5 isoform X2 [KO:K13342]
102519081  PEX5L; LOW QUALITY PROTEIN: PEX5-related protein [KO:K13342]
102509892  PEX14; peroxisomal membrane protein PEX14 [KO:K13343]
102504291  PEX13; peroxisome biogenesis factor 13 isoform X1 [KO:K13344]
102522338  PEX12; peroxisome assembly protein 12 isoform X2 [KO:K13345]
102505128  PEX10; peroxisome biogenesis factor 10 isoform X1 [KO:K13346]
102503509  PEX2; peroxisome biogenesis factor 2 [KO:K06664]
102510029  PXMP2; peroxisomal membrane protein 2 isoform X1 [KO:K13347]
102515470  LOW QUALITY PROTEIN: peroxisomal membrane protein 2-like [KO:K13347]
102513693  MPV17L2; mpv17-like protein 2 [KO:K13348]
102514175  MPV17; protein Mpv17 isoform X2 [KO:K13348]
102514697  MPV17L; mpv17-like protein isoform X1 [KO:K13349]
102511003  PXMP4; peroxisomal membrane protein 4 isoform X2 [KO:K13350]
102508011  PEX11A; peroxisomal membrane protein 11A isoform X1 [KO:K13351]
102518946  PEX11B; peroxisomal membrane protein 11B [KO:K13352]
102509557  PEX11G; peroxisomal membrane protein 11C [KO:K13353]
102517071  SLC25A17; peroxisomal membrane protein PMP34 isoform X2 [KO:K13354]
102508961  HACL1; 2-hydroxyacyl-CoA lyase 1 isoform X1 [KO:K12261] [EC:4.1.2.63]
102518501  AMACR; alpha-methylacyl-CoA racemase [KO:K01796] [EC:5.1.99.4]
102511029  PHYH; phytanoyl-CoA dioxygenase, peroxisomal [KO:K00477] [EC:1.14.11.18]
102515875  ACOX3; LOW QUALITY PROTEIN: peroxisomal acyl-coenzyme A oxidase 3 [KO:K00232] [EC:1.3.3.6]
102512004  ACOX1; peroxisomal acyl-coenzyme A oxidase 1 isoform X2 [KO:K00232] [EC:1.3.3.6]
102512694  ACOX2; LOW QUALITY PROTEIN: peroxisomal acyl-coenzyme A oxidase 2 [KO:K10214] [EC:1.17.99.3]
102508317  HSD17B4; peroxisomal multifunctional enzyme type 2 [KO:K12405] [EC:1.1.1.- 4.2.1.107 4.2.1.119]
102516689  SCP2; non-specific lipid-transfer protein [KO:K08764] [EC:2.3.1.176]
102523415  BAAT; bile acid-CoA:amino acid N-acyltransferase isoform X3 [KO:K00659] [EC:2.3.1.65 3.1.2.2]
102519802  EHHADH; peroxisomal bifunctional enzyme [KO:K07514] [EC:4.2.1.17 1.1.1.35 5.3.3.8]
102506838  ACAA1; 3-ketoacyl-CoA thiolase, peroxisomal [KO:K07513] [EC:2.3.1.16]
102522579  DECR2; peroxisomal 2,4-dienoyl-CoA reductase isoform X8 [KO:K13237] [EC:1.3.1.124]
102520180  ECH1; delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, mitochondrial [KO:K12663] [EC:5.3.3.21]
102507508  ABCD1; ATP-binding cassette sub-family D member 1 [KO:K05675] [EC:7.6.2.4]
102510221  ABCD2; ATP-binding cassette sub-family D member 2 [KO:K05676] [EC:7.6.2.4]
102523326  ABCD4; ATP-binding cassette sub-family D member 4 isoform X4 [KO:K05678] [EC:7.6.2.8]
102514979  SLC27A2; very long-chain acyl-CoA synthetase [KO:K08746] [EC:6.2.1.- 6.2.1.3]
102523503  ACSL6; long-chain-fatty-acid--CoA ligase 6 isoform X6 [KO:K01897] [EC:6.2.1.3]
102507504  ACSL1; long-chain-fatty-acid--CoA ligase 1 isoform X1 [KO:K01897] [EC:6.2.1.3]
102513167  ACSL4; long-chain-fatty-acid--CoA ligase 4 isoform X2 [KO:K01897] [EC:6.2.1.3]
102512123  ACSL5; long-chain-fatty-acid--CoA ligase 5 [KO:K01897] [EC:6.2.1.3]
102507791  ACSL3; long-chain-fatty-acid--CoA ligase 3 [KO:K01897] [EC:6.2.1.3]
102511162  PECR; peroxisomal trans-2-enoyl-CoA reductase isoform X2 [KO:K07753] [EC:1.3.1.38]
102523911  ECI2; enoyl-CoA delta isomerase 2, mitochondrial isoform X1 [KO:K13239] [EC:5.3.3.8]
102503968  NUDT7; peroxisomal coenzyme A diphosphatase NUDT7 [KO:K17879] [EC:3.6.1.77]
102510854  NUDT12; peroxisomal NADH pyrophosphatase NUDT12 [KO:K03426] [EC:3.6.1.22]
102511331  NUDT13; nucleoside diphosphate-linked moiety X motif 13 isoform X1 [KO:K03426] [EC:3.6.1.22]
102508395  NUDT19; nucleoside diphosphate-linked moiety X motif 19 [KO:K13355] [EC:3.6.1.77]
102506493  ACOT8; acyl-coenzyme A thioesterase 8 [KO:K11992] [EC:3.1.2.27]
102506902  CRAT; carnitine O-acetyltransferase isoform X1 [KO:K00624] [EC:2.3.1.7]
102512006  CROT; peroxisomal carnitine O-octanoyltransferase [KO:K05940] [EC:2.3.1.137]
102519722  MLYCD; LOW QUALITY PROTEIN: malonyl-CoA decarboxylase, mitochondrial [KO:K01578] [EC:4.1.1.9]
102522815  GNPAT; dihydroxyacetone phosphate acyltransferase [KO:K00649] [EC:2.3.1.42]
102507465  AGPS; alkyldihydroxyacetonephosphate synthase, peroxisomal [KO:K00803] [EC:2.5.1.26]
102522950  FAR1; fatty acyl-CoA reductase 1 isoform X1 [KO:K13356] [EC:1.2.1.84]
102505508  FAR2; fatty acyl-CoA reductase 2 isoform X1 [KO:K13356] [EC:1.2.1.84]
102510881  MVK; mevalonate kinase isoform X1 [KO:K00869] [EC:2.7.1.36]
102523720  PMVK; phosphomevalonate kinase isoform X1 [KO:K13273] [EC:2.7.4.2]
102506707  AGXT; serine--pyruvate aminotransferase [KO:K00830] [EC:2.6.1.44 2.6.1.45 2.6.1.51]
102513211  DAO; D-amino-acid oxidase [KO:K00273] [EC:1.4.3.3]
102510004  DDO; D-aspartate oxidase [KO:K00272] [EC:1.4.3.1]
102506545  IDH1; isocitrate dehydrogenase [NADP] cytoplasmic [KO:K00031] [EC:1.1.1.42]
102509802  IDH2; isocitrate dehydrogenase [NADP], mitochondrial [KO:K00031] [EC:1.1.1.42]
102509887  PAOX; peroxisomal N(1)-acetyl-spermine/spermidine oxidase isoform X2 [KO:K00308] [EC:1.5.3.13]
102505288  PIPOX; peroxisomal sarcosine oxidase isoform X1 [KO:K00306] [EC:1.5.3.1 1.5.3.7]
102516003  HMGCL; hydroxymethylglutaryl-CoA lyase, mitochondrial isoform X1 [KO:K01640] [EC:4.1.3.4]
102521399  HMGCLL1; 3-hydroxymethyl-3-methylglutaryl-CoA lyase, cytoplasmic isoform X1 [KO:K01640] [EC:4.1.3.4]
102512205  HAO2; hydroxyacid oxidase 2 isoform X1 [KO:K11517] [EC:1.1.3.15]
102514400  HAO1; hydroxyacid oxidase 1 isoform X1 [KO:K11517] [EC:1.1.3.15]
102524165  CAT; catalase [KO:K03781] [EC:1.11.1.6]
102514564  PRDX5; peroxiredoxin-5, mitochondrial isoform X1 [KO:K11187] [EC:1.11.1.24]
102505386  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
102504600  LOW QUALITY PROTEIN: superoxide dismutase [Cu-Zn]-like [KO:K04565] [EC:1.15.1.1]
102522368  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
102518523  NOS2; nitric oxide synthase, inducible isoform X1 [KO:K13241] [EC:1.14.13.39]
102508982  PRDX1; LOW QUALITY PROTEIN: peroxiredoxin-1 [KO:K13279] [EC:1.11.1.24]
102517269  EPHX2; bifunctional epoxide hydrolase 2 [KO:K08726] [EC:3.3.2.10 3.1.3.76]
102518852  GSTK1; glutathione S-transferase kappa 1 [KO:K13299] [EC:2.5.1.18]
102523581  XDH; xanthine dehydrogenase/oxidase isoform X1 [KO:K00106] [EC:1.17.1.4 1.17.3.2]
102516222  dehydrogenase/reductase SDR family member 4-like 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
cfr00071  Fatty acid degradation
cfr00120  Primary bile acid biosynthesis
cfr00230  Purine metabolism
cfr00564  Glycerophospholipid metabolism
cfr00565  Ether lipid metabolism
cfr00830  Retinol metabolism
cfr00900  Terpenoid backbone biosynthesis
cfr03320  PPAR signaling pathway
KO pathway
ko04146   
LinkDB

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