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 K00031 IDH1, IDH2, icd, IDP; isocitrate dehydrogenase [EC:1.1.1.42] K00106 XDH; xanthine dehydrogenase/oxidase [EC:1.17.1.4 1.17.3.2] K00232 ACOX1_3, POX1; acyl-CoA oxidase [EC:1.3.3.6] K00272 DDO; D-aspartate oxidase [EC:1.4.3.1] K00273 DAO, aao; D-amino-acid oxidase [EC:1.4.3.3] K00306 PIPOX; sarcosine oxidase / L-pipecolate oxidase [EC:1.5.3.1 1.5.3.7] K00308 PAOX; N1-acetylpolyamine oxidase [EC:1.5.3.13] K00477 PHYH; phytanoyl-CoA hydroxylase [EC:1.14.11.18] K00624 CRAT; carnitine O-acetyltransferase [EC:2.3.1.7] K00649 GNPAT; glyceronephosphate O-acyltransferase [EC:2.3.1.42] K00659 BAAT; bile acid-CoA:amino acid N-acyltransferase [EC:2.3.1.65 3.1.2.27 3.1.2.2] K00803 AGPS; alkyldihydroxyacetonephosphate synthase [EC:2.5.1.26] K00830 AGXT; alanine-glyoxylate transaminase / serine-glyoxylate transaminase / serine-pyruvate transaminase [EC:2.6.1.44 2.6.1.45 2.6.1.51] K00869 MVK, mvaK1; mevalonate kinase [EC:2.7.1.36] K01578 MLYCD; malonyl-CoA decarboxylase [EC:4.1.1.9] K01640 HMGCL, hmgL; hydroxymethylglutaryl-CoA lyase [EC:4.1.3.4] K01796 AMACR, mcr; alpha-methylacyl-CoA racemase [EC:5.1.99.4] K01897 ACSL, fadD; long-chain-fatty-acid---CoA ligase [EC:6.2.1.3] K03426 NUDT12_13, nudC; NAD+ diphosphatase [EC:3.6.1.22] K03781 katE, CAT, catB, srpA; catalase [EC:1.11.1.6] K04564 SOD2; superoxide dismutase, Fe-Mn family [EC:1.15.1.1] K04565 SOD1; superoxide dismutase, Cu-Zn family [EC:1.15.1.1] K05675 ABCD1, ALD; ATP-binding cassette, subfamily D (ALD), member 1 [EC:7.6.2.4] K05676 ABCD2, ALDL1; ATP-binding cassette, subfamily D (ALD), member 2 [EC:7.6.2.4] K05677 ABCD3, PMP70; ATP-binding cassette, subfamily D (ALD), member 3 [EC:7.6.2.4] K05678 ABCD4, PXMP1L; ATP-binding cassette, subfamily D (ALD), member 4 [EC:7.6.2.8] K05940 CROT; carnitine O-octanoyltransferase [EC:2.3.1.137] K06664 PEX2, PXMP3; peroxin-2 [EC:2.3.2.27 2.3.2.36] K07513 ACAA1, POT1; acetyl-CoA acyltransferase 1 [EC:2.3.1.16] K07514 EHHADH; 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] K07753 PECR; peroxisomal trans-2-enoyl-CoA reductase [EC:1.3.1.38] K08726 EPHX2; soluble epoxide hydrolase / lipid-phosphate phosphatase [EC:3.3.2.10 3.1.3.76] K08746 SLC27A2, FACVL1, FATP2; solute carrier family 27 (fatty acid transporter), member 2 [EC:6.2.1.- 6.2.1.3] K08764 SCP2, SCPX; sterol carrier protein 2 [EC:2.3.1.176] K10214 ACOX2; 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoyl-CoA 24-hydroxylase [EC:1.17.99.3] K11147 DHRS4; dehydrogenase/reductase SDR family member 4 [EC:1.1.-.-] K11187 PRDX5; peroxiredoxin 5 [EC:1.11.1.24] K11517 HAO; (S)-2-hydroxy-acid oxidase [EC:1.1.3.15] K11992 ACOT8, PTE; acyl-CoA thioesterase 8 [EC:3.1.2.27] K12261 HACL1; 2-hydroxyacyl-CoA lyase [EC:4.1.2.63] K12405 HSD17B4; (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] K12663 ECH1; Delta3,5-Delta2,4-dienoyl-CoA isomerase [EC:5.3.3.21] K13237 DECR2, SPS19; 2,4-dienoyl-CoA reductase [(3E)-enoyl-CoA-producing], peroxisomal [EC:1.3.1.124] K13239 ECI2, PECI, ECI1; Delta3-Delta2-enoyl-CoA isomerase [EC:5.3.3.8] K13241 NOS2; nitric-oxide synthase, inducible [EC:1.14.13.39] K13273 PMVK; phosphomevalonate kinase [EC:2.7.4.2] K13279 PRDX1; peroxiredoxin 1 [EC:1.11.1.24] K13299 GSTK1; glutathione S-transferase kappa 1 [EC:2.5.1.18] K13335 PEX16; peroxin-16 K13336 PEX3; peroxin-3 K13337 PEX19; peroxin-19 K13338 PEX1; peroxin-1 [EC:3.6.4.-] K13339 PEX6, PXAAA1; peroxin-6 [EC:3.6.4.-] K13340 PEX26; peroxin-26 K13341 PEX7, PTS2R; peroxin-7 K13342 PEX5, PXR1; peroxin-5 K13343 PEX14; peroxin-14 K13344 PEX13; peroxin-13 K13345 PEX12, PAF3; peroxin-12 K13346 PEX10; peroxin-10 [EC:2.3.2.27] K13347 PXMP2, PMP22; peroxisomal membrane protein 2 K13348 MPV17; protein Mpv17 K13349 MPV17L; Mpv17-like protein K13350 PXMP4, PMP24; peroxisomal membrane protein 4 K13351 PEX11A; peroxin-11A K13352 PEX11B; peroxin-11B K13353 PEX11C; peroxin-11C K13354 SLC25A17, PMP34; solute carrier family 25 (peroxisomal adenine nucleotide transporter), member 17 K13355 NUDT19; acyl-coenzyme A diphosphatase NUDT19, mitochondrial [EC:3.6.1.77] K13356 FAR; alcohol-forming fatty acyl-CoA reductase [EC:1.2.1.84] K15628 PXA; ATP-binding cassette, subfamily D (ALD), peroxisomal long-chain fatty acid import protein [EC:7.6.2.4] K17879 NUDT7; peroxisomal coenzyme A diphosphatase NUDT7 [EC:3.6.1.77] K22587 DCI1; Delta3,5-Delta2,4-dienoyl-CoA isomerase [EC:5.3.3.21] K25819 ABCD1, PXA1; ATP-binding cassette, subfamily D (ALD), peroxisomal ABC transporter 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 ///