KEGG   PATHWAY: ko04146Help
Entry
ko04146                     Pathway                                

Name
Peroxisome
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
BRITE hierarchy
Pathway map
ko04146  Peroxisome
ko04146

Ortholog table
Disease
H00075  Refsum disease
H00176  Adrenoleukodystrophy
H00177  Neonatal adrenoleukodystrophy
H00203  Acatalasemia
H00205  Peroxisome biogenesis disorder
H00207  Rhizomelic chondrodysplasia punctata
H00407  Peroxisomal beta-oxidation enzyme deficiency
H00628  Congenital bile acid synthesis defect
H00874  Leukoencephalopathy with dystonia and motor neuropathy
H01155  Roussy-Levy syndrome
H01283  Malonyl-CoA decarboxylase deficiency
H01342  Zellweger syndrome
H02096  Peroxisomal acyl-CoA oxidase deficiency
H02098  D-bifunctional protein deficiency
H02099  Alpha-methylacyl-CoA racemase deficiency
H02100  Peroxisomal fatty acyl-CoA reductase 1 disorder
Other DBs
GO: 0005777
Orthology
K13335  peroxin-16
K13336  peroxin-3
K13337  peroxin-19
K05677  ATP-binding cassette, subfamily D (ALD), member 3
K13338  peroxin-1
K13339  peroxin-6
K13340  peroxin-26
K13341  peroxin-7
K13342  peroxin-5
K13343  peroxin-14
K13344  peroxin-13
K13345  peroxin-12
K13346  peroxin-10
K06664  peroxin-2
K13347  peroxisomal membrane protein 2
K13348  protein Mpv17
K13349  Mpv17-like protein
K13350  peroxisomal membrane protein 4
K13351  peroxin-11A
K13352  peroxin-11B
K13353  peroxin-11C
K13354  solute carrier family 25 (peroxisomal adenine nucleotide transporter), member 17
K12261  2-hydroxyacyl-CoA lyase 1 [EC:4.1.-.-]
K01796  alpha-methylacyl-CoA racemase [EC:5.1.99.4]
K00477  phytanoyl-CoA hydroxylase [EC:1.14.11.18]
K00232  acyl-CoA oxidase [EC:1.3.3.6]
K10214  3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase [EC:1.17.99.3]
K12405  (3R)-3-hydroxyacyl-CoA dehydrogenase / 3a,7a,12a-trihydroxy-5b-cholest-24-enoyl-CoA hydratase / enoyl-CoA hydratase 2 [EC:1.1.1.- 4.2.1.107 4.2.1.119]
K08764  sterol carrier protein 2 [EC:2.3.1.176]
K00659  bile acid-CoA:amino acid N-acyltransferase [EC:2.3.1.65 3.1.2.2]
K07514  enoyl-CoA hydratase / 3-hydroxyacyl-CoA dehydrogenase / 3,2-trans-enoyl-CoA isomerase [EC:4.2.1.17 1.1.1.35 5.3.3.8]
K07513  acetyl-CoA acyltransferase 1 [EC:2.3.1.16]
K13237  peroxisomal 2,4-dienoyl-CoA reductase [EC:1.3.1.34]
K12663  Delta3,5-Delta2,4-dienoyl-CoA isomerase [EC:5.3.3.21]
K05675  ATP-binding cassette, subfamily D (ALD), member 1
K05676  ATP-binding cassette, subfamily D (ALD), member 2
K05678  ATP-binding cassette, subfamily D (ALD), member 4
K15628  ATP-binding cassette, subfamily D (ALD), peroxisomal long-chain fatty acid import protein
K08746  solute carrier family 27 (fatty acid transporter), member 2 [EC:6.2.1.- 6.2.1.3]
K01897  long-chain acyl-CoA synthetase [EC:6.2.1.3]
K07753  peroxisomal trans-2-enoyl-CoA reductase [EC:1.3.1.38]
K13239  Delta3-Delta2-enoyl-CoA isomerase [EC:5.3.3.8]
K22587  Delta3,5-Delta2,4-dienoyl-CoA isomerase [EC:5.3.3.21]
K17879  peroxisomal coenzyme A diphosphatase NUDT7 [EC:3.6.1.-]
K03426  NAD+ diphosphatase [EC:3.6.1.22]
K13355  nucleoside diphosphate-linked moiety X motif 19, mitochondrial [EC:3.6.1.-]
K11992  acyl-CoA thioesterase 8 [EC:3.1.2.27]
K00624  carnitine O-acetyltransferase [EC:2.3.1.7]
K05940  carnitine O-octanoyltransferase [EC:2.3.1.137]
K01578  malonyl-CoA decarboxylase [EC:4.1.1.9]
K00649  glyceronephosphate O-acyltransferase [EC:2.3.1.42]
K00803  alkyldihydroxyacetonephosphate synthase [EC:2.5.1.26]
K13356  alcohol-forming fatty acyl-CoA reductase [EC:1.2.1.84]
K00869  mevalonate kinase [EC:2.7.1.36]
K13273  phosphomevalonate kinase [EC:2.7.4.2]
K00830  alanine-glyoxylate transaminase / serine-glyoxylate transaminase / serine-pyruvate transaminase [EC:2.6.1.44 2.6.1.45 2.6.1.51]
K00273  D-amino-acid oxidase [EC:1.4.3.3]
K00272  D-aspartate oxidase [EC:1.4.3.1]
K00031  isocitrate dehydrogenase [EC:1.1.1.42]
K00308  N1-acetylpolyamine oxidase [EC:1.5.3.13]
K00306  sarcosine oxidase / L-pipecolate oxidase [EC:1.5.3.1 1.5.3.7]
K01640  hydroxymethylglutaryl-CoA lyase [EC:4.1.3.4]
K11517  (S)-2-hydroxy-acid oxidase [EC:1.1.3.15]
K03781  catalase [EC:1.11.1.6]
K11187  peroxiredoxin 5, atypical 2-Cys peroxiredoxin [EC:1.11.1.15]
K04565  superoxide dismutase, Cu-Zn family [EC:1.15.1.1]
K04564  superoxide dismutase, Fe-Mn family [EC:1.15.1.1]
K13241  nitric-oxide synthase, inducible [EC:1.14.13.39]
K13279  peroxiredoxin 1 [EC:1.11.1.15]
K08726  soluble epoxide hydrolase / lipid-phosphate phosphatase [EC:3.3.2.10 3.1.3.76]
K13299  glutathione S-transferase kappa 1 [EC:2.5.1.18]
K00106  xanthine dehydrogenase/oxidase [EC:1.17.1.4 1.17.3.2]
K11147  dehydrogenase/reductase SDR family member 4 [EC:1.1.-.-]
Reference
  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
  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
  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
  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
Reference
  Authors
Kleinman R, Goulet OJ, Mieli-Vergani G, Sanderson I, Sherman P, Shneider B.
  Title
Walker's Pediatric Gastrointestinal Disease (5th edition)
  Journal
B.C.Decker (2008)
Related
pathway
ko00071  Fatty acid degradation
ko00120  Primary bile acid biosynthesis
ko00230  Purine metabolism
ko00564  Glycerophospholipid metabolism
ko00565  Ether lipid metabolism
ko00830  Retinol metabolism
ko00900  Terpenoid backbone biosynthesis
ko03320  PPAR signaling pathway
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