KEGG   PATHWAY: mlf04146
Entry
mlf04146                    Pathway                                
Name
Peroxisome - Myotis lucifugus (little brown bat)
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
mlf04146  Peroxisome
mlf04146

Other DBs
GO: 0005777
Organism
Myotis lucifugus (little brown bat) [GN:mlf]
Gene
102435338  PEX16; peroxisomal biogenesis factor 16 isoform X2 [KO:K13335]
102433940  PEX3; peroxisomal biogenesis factor 3 isoform X1 [KO:K13336]
102443121  PEX19; peroxisomal biogenesis factor 19 [KO:K13337]
102430976  ABCD3; ATP-binding cassette sub-family D member 3 isoform X1 [KO:K05677] [EC:7.6.2.4]
102428438  PEX1; peroxisome biogenesis factor 1 isoform X1 [KO:K13338]
102442709  PEX6; LOW QUALITY PROTEIN: peroxisome biogenesis factor 6 [KO:K13339]
102425305  PEX26; peroxisome assembly protein 26 isoform X1 [KO:K13340]
102440961  PEX7; peroxisomal biogenesis factor 7 isoform X1 [KO:K13341]
102428130  PEX5L; PEX5-related protein [KO:K13342]
102435184  PEX5; peroxisomal biogenesis factor 5 isoform X1 [KO:K13342]
102435046  peroxisomal targeting signal 1 receptor-like isoform X1 [KO:K13342]
102429661  PEX14; peroxisomal membrane protein PEX14 isoform X1 [KO:K13343]
102432676  PEX13; peroxisome biogenesis factor 13 [KO:K13344]
102441665  PEX12; peroxisome assembly protein 12 [KO:K13345]
102438998  PEX10; LOW QUALITY PROTEIN: peroxisome biogenesis factor 10 [KO:K13346]
102429563  PEX2; peroxisome biogenesis factor 2 [KO:K06664]
102441391  PXMP2; peroxisomal membrane protein 2 [KO:K13347]
102441345  MPV17; protein Mpv17 isoform X1 [KO:K13348]
102430351  MPV17L2; mpv17-like protein 2 [KO:K13348]
102422945  mpv17-like protein isoform X1 [KO:K13349]
102424194  PXMP4; peroxisomal membrane protein 4 [KO:K13350]
102421980  PEX11A; peroxisomal membrane protein 11A [KO:K13351]
102423207  PEX11B; peroxisomal membrane protein 11B [KO:K13352]
102436426  PEX11G; LOW QUALITY PROTEIN: peroxisomal membrane protein 11C [KO:K13353]
102432763  SLC25A17; peroxisomal membrane protein PMP34 [KO:K13354]
102419042  HACL1; 2-hydroxyacyl-CoA lyase 1 isoform X1 [KO:K12261] [EC:4.1.2.63]
102422682  AMACR; LOW QUALITY PROTEIN: alpha-methylacyl-CoA racemase [KO:K01796] [EC:5.1.99.4]
102418211  PHYH; phytanoyl-CoA dioxygenase, peroxisomal [KO:K00477] [EC:1.14.11.18]
102426347  ACOX1; peroxisomal acyl-coenzyme A oxidase 1 isoform X1 [KO:K00232] [EC:1.3.3.6]
102428739  ACOX2; peroxisomal acyl-coenzyme A oxidase 2 isoform X2 [KO:K10214] [EC:1.17.99.3]
102425700  HSD17B4; peroxisomal multifunctional enzyme type 2 [KO:K12405] [EC:1.1.1.- 4.2.1.107 4.2.1.119]
102427215  SCP2; non-specific lipid-transfer protein [KO:K08764] [EC:2.3.1.176]
102433860  acyl-coenzyme A amino acid N-acyltransferase 2-like isoform X2 [KO:K00659] [EC:2.3.1.65 3.1.2.2]
102434162  BAAT; bile acid-CoA:amino acid N-acyltransferase [KO:K00659] [EC:2.3.1.65 3.1.2.2]
102438879  EHHADH; peroxisomal bifunctional enzyme isoform X1 [KO:K07514] [EC:4.2.1.17 1.1.1.35 5.3.3.8]
102425698  ACAA1; 3-ketoacyl-CoA thiolase, peroxisomal [KO:K07513] [EC:2.3.1.16]
102419764  DECR2; peroxisomal 2,4-dienoyl-CoA reductase [KO:K13237] [EC:1.3.1.124]
102442386  ECH1; delta(3,5)-Delta(2,4)-dienoyl-CoA isomerase, mitochondrial [KO:K12663] [EC:5.3.3.21]
102425113  ABCD1; LOW QUALITY PROTEIN: ATP-binding cassette sub-family D member 1 [KO:K05675] [EC:7.6.2.4]
102441183  ABCD2; ATP-binding cassette sub-family D member 2 isoform X1 [KO:K05676] [EC:7.6.2.4]
102424744  ABCD4; LOW QUALITY PROTEIN: ATP-binding cassette sub-family D member 4 [KO:K05678] [EC:7.6.2.8]
102422949  SLC27A2; very long-chain acyl-CoA synthetase [KO:K08746] [EC:6.2.1.- 6.2.1.3]
102439168  ACSL3; long-chain-fatty-acid--CoA ligase 3 isoform X1 [KO:K01897] [EC:6.2.1.3]
102426690  ACSL5; long-chain-fatty-acid--CoA ligase 5 isoform X1 [KO:K01897] [EC:6.2.1.3]
102437099  ACSL1; long-chain-fatty-acid--CoA ligase 1 isoform X4 [KO:K01897] [EC:6.2.1.3]
102443032  ACSL4; long-chain-fatty-acid--CoA ligase 4 [KO:K01897] [EC:6.2.1.3]
102427269  ACSL6; long-chain-fatty-acid--CoA ligase 6 [KO:K01897] [EC:6.2.1.3]
102440751  PECR; peroxisomal trans-2-enoyl-CoA reductase isoform X1 [KO:K07753] [EC:1.3.1.38]
102441038  ECI2; enoyl-CoA delta isomerase 2, mitochondrial isoform X1 [KO:K13239] [EC:5.3.3.8]
102425413  NUDT7; peroxisomal coenzyme A diphosphatase NUDT7 [KO:K17879] [EC:3.6.1.77]
102419745  NUDT12; peroxisomal NADH pyrophosphatase NUDT12 isoform X1 [KO:K03426] [EC:3.6.1.22]
102417609  NUDT13; nucleoside diphosphate-linked moiety X motif 13 isoform X1 [KO:K03426] [EC:3.6.1.22]
102427254  NUDT19; LOW QUALITY PROTEIN: nucleoside diphosphate-linked moiety X motif 19 [KO:K13355] [EC:3.6.1.77]
102421081  ACOT8; acyl-coenzyme A thioesterase 8 [KO:K11992] [EC:3.1.2.27]
102433728  CRAT; carnitine O-acetyltransferase isoform X1 [KO:K00624] [EC:2.3.1.7]
102422510  CROT; peroxisomal carnitine O-octanoyltransferase isoform X1 [KO:K05940] [EC:2.3.1.137]
102420842  GNPAT; dihydroxyacetone phosphate acyltransferase isoform X1 [KO:K00649] [EC:2.3.1.42]
102440334  AGPS; alkyldihydroxyacetonephosphate synthase, peroxisomal isoform X1 [KO:K00803] [EC:2.5.1.26]
102422457  FAR2; fatty acyl-CoA reductase 2 [KO:K13356] [EC:1.2.1.84]
102427013  FAR1; fatty acyl-CoA reductase 1 isoform X2 [KO:K13356] [EC:1.2.1.84]
102436232  MVK; mevalonate kinase [KO:K00869] [EC:2.7.1.36]
102431912  PMVK; phosphomevalonate kinase [KO:K13273] [EC:2.7.4.2]
102416821  AGXT; serine--pyruvate aminotransferase [KO:K00830] [EC:2.6.1.44 2.6.1.45 2.6.1.51]
102432979  DAO; D-amino-acid oxidase [KO:K00273] [EC:1.4.3.3]
102426872  DDO; D-aspartate oxidase isoform X1 [KO:K00272] [EC:1.4.3.1]
102432786  IDH1; isocitrate dehydrogenase [NADP] cytoplasmic isoform X3 [KO:K00031] [EC:1.1.1.42]
102421663  IDH2; isocitrate dehydrogenase [NADP], mitochondrial [KO:K00031] [EC:1.1.1.42]
102426319  PAOX; LOW QUALITY PROTEIN: peroxisomal N(1)-acetyl-spermine/spermidine oxidase [KO:K00308] [EC:1.5.3.13]
102434938  PIPOX; peroxisomal sarcosine oxidase [KO:K00306] [EC:1.5.3.1 1.5.3.7]
102421588  HMGCL; hydroxymethylglutaryl-CoA lyase, mitochondrial isoform X7 [KO:K01640] [EC:4.1.3.4]
102433053  HMGCLL1; 3-hydroxymethyl-3-methylglutaryl-CoA lyase, cytoplasmic isoform X1 [KO:K01640] [EC:4.1.3.4]
102421694  HAO2; hydroxyacid oxidase 2 [KO:K11517] [EC:1.1.3.15]
102428306  HAO1; hydroxyacid oxidase 1 [KO:K11517] [EC:1.1.3.15]
102422808  CAT; LOW QUALITY PROTEIN: catalase [KO:K03781] [EC:1.11.1.6]
102434903  PRDX5; peroxiredoxin-5, mitochondrial [KO:K11187] [EC:1.11.1.24]
102435629  LOW QUALITY PROTEIN: peroxiredoxin-5, mitochondrial-like [KO:K11187] [EC:1.11.1.24]
102424909  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
102424669  SOD2; superoxide dismutase [Mn], mitochondrial isoform X1 [KO:K04564] [EC:1.15.1.1]
102424107  NOS2; LOW QUALITY PROTEIN: nitric oxide synthase, inducible [KO:K13241] [EC:1.14.13.39]
102430863  PRDX1; peroxiredoxin-1 [KO:K13279] [EC:1.11.1.24]
102426648  EPHX2; bifunctional epoxide hydrolase 2 [KO:K08726] [EC:3.3.2.10 3.1.3.76]
102429821  GSTK1; glutathione S-transferase kappa 1 isoform X1 [KO:K13299] [EC:2.5.1.18]
102441222  XDH; xanthine dehydrogenase/oxidase isoform X2 [KO:K00106] [EC:1.17.1.4 1.17.3.2]
102431416  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
mlf00071  Fatty acid degradation
mlf00120  Primary bile acid biosynthesis
mlf00230  Purine metabolism
mlf00564  Glycerophospholipid metabolism
mlf00565  Ether lipid metabolism
mlf00830  Retinol metabolism
mlf00900  Terpenoid backbone biosynthesis
mlf03320  PPAR signaling pathway
KO pathway
ko04146   
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