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 PATHWAY_MAP ko04146 Peroxisome DBLINKS GO: 0005777 ORTHOLOGY K13335 peroxin-16 K13336 peroxin-3 K13337 peroxin-19 K05677 ATP-binding cassette, subfamily D (ALD), member 3 [EC:7.6.2.4] 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 [EC:4.1.2.63] 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 2,4-dienoyl-CoA reductase [(3E)-enoyl-CoA-producing], peroxisomal [EC:1.3.1.124] K12663 Delta3,5-Delta2,4-dienoyl-CoA isomerase [EC:5.3.3.21] K05675 ATP-binding cassette, subfamily D (ALD), member 1 [EC:7.6.2.4] K05676 ATP-binding cassette, subfamily D (ALD), member 2 [EC:7.6.2.4] K05678 ATP-binding cassette, subfamily D (ALD), member 4 [EC:7.6.2.8] K25819 ATP-binding cassette, subfamily D (ALD), peroxisomal ABC transporter [EC:7.6.2.4] K15628 ATP-binding cassette, subfamily D (ALD), peroxisomal long-chain fatty acid import protein [EC:7.6.2.4] 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:3.6.1.77] K03426 NAD+ diphosphatase [EC:3.6.1.22] K13355 acyl-coenzyme A diphosphatase NUDT19, mitochondrial [EC:3.6.1.77] 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 [EC:1.11.1.24] 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:1.11.1.24] 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 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 REL_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 ///