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Details on Person UniProt:P55096 Abcd3
| Class:Id | ReferenceGeneProduct:84381 |
|---|---|
| _chainChangeLog | initiator methionine:1 added on Sat February 7 2015;chain:2-659 added on Sat February 7 2015 |
| _displayName | UniProt:P55096 Abcd3 |
| _timestamp | 2025-02-21 19:35:05 |
| chain | chain:1-659 |
| checksum | 6DF924D91B481DC4 |
| comment | FUNCTION Broad substrate specificity ATP-dependent transporter of the ATP-binding cassette (ABC) family that catalyzes the transport of long-chain fatty acids (LCFA)-CoA, dicarboxylic acids-CoA, long-branched-chain fatty acids-CoA and bile acids from the cytosol to the peroxisome lumen for beta-oxydation. Has fatty acyl-CoA thioesterase and ATPase activities (By similarity). Probably hydrolyzes fatty acyl-CoAs into free fatty acids prior to their ATP-dependent transport into peroxisomes (By similarity). Thus, play a role in regulation of LCFAs and energy metabolism namely, in the degradation and biosynthesis of fatty acids by beta-oxidation (PubMed:25168382).CATALYTIC ACTIVITY a very long-chain fatty acyl-CoA + H2O = a very long-chain fatty acid + CoA + H(+)CATALYTIC ACTIVITY a very long-chain fatty acid(in) + ATP + H2O = a very long-chain fatty acid(out) + ADP + phosphate + H(+)CATALYTIC ACTIVITY a long-chain fatty acyl-CoA + H2O = a long-chain fatty acid + CoA + H(+)CATALYTIC ACTIVITY a long-chain fatty acid(in) + ATP + H2O = a long-chain fatty acid(out) + ADP + phosphate + H(+)CATALYTIC ACTIVITY pristanoyl-CoA + H2O = 2,6,10,14-tetramethylpentadecanoate + CoA + H(+)CATALYTIC ACTIVITY 2,6,10,14-tetramethylpentadecanoate(in) + ATP + H2O = 2,6,10,14-tetramethylpentadecanoate(out) + ADP + phosphate + H(+)CATALYTIC ACTIVITY hexadecanedioyl-CoA + H2O = hexadecanedioate + CoA + H(+)CATALYTIC ACTIVITY hexadecanedioate(in) + ATP + H2O = hexadecanedioate(out) + ADP + phosphate + H(+)CATALYTIC ACTIVITY (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA + H2O = (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoate + CoA + H(+)CATALYTIC ACTIVITY (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoate(in) + ATP + H2O = (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoate(out) + ADP + phosphate + H(+)CATALYTIC ACTIVITY (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA + H2O = (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate + CoA + H(+)CATALYTIC ACTIVITY (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate(in) + ATP + H2O = (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate(out) + ADP + phosphate + H(+)SUBUNIT Homodimers. Can form heterodimers with ABCD1 and ABCD2. Dimerization is necessary to form an active transporter. Interacts with PEX19; mediates the targeting of ABCD3 to peroxisomes.SUBCELLULAR LOCATION Ubiquitinated by PEX2 during pexophagy in response to starvation, leading to its degradation.DISRUPTION PHENOTYPE Animals show normal activity and have no obvious malformations. However, necroscopy show increased liver size compared to controls, and fibroblasts show reduced numbers of enlarged peroxisomes. They show however a defect in bile acid biosynthesis and they have impaired beta-oxidation of the branched-chain pristanic and phytanic fatty acids on a phytol-loaded diet.SIMILARITY Belongs to the ABC transporter superfamily. ABCD family. Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily. |
| description | recommendedName: ATP-binding cassette sub-family D member 3 ecNumber evidence="1"3.1.2.- ecNumber evidence="1"7.6.2.- alternativeName: 68 kDa peroxisomal membrane protein shortName: PMP68 alternativeName: 70 kDa peroxisomal membrane protein shortName: PMP70 |
| geneName | Abcd3 Pmp70 Pxmp1 |
| identifier | P55096 |
| isSequenceChanged | FALSE |
| keyword | Acetylation ATP-binding Glycoprotein Hydrolase Membrane Nucleotide-binding Peroxisome Phosphoprotein Reference proteome Translocase Transmembrane Transmembrane helix Transport Ubl conjugation |
| modified | [InstanceEdit:143527] Schmidt, EE, 2004-11-12 07:45:10 [InstanceEdit:217385] Schmidt, EE, 2008-03-27 06:23:53 [InstanceEdit:354386] Schmidt, EE, 2008-06-18 04:45:12 [InstanceEdit:384350] Kanapin, AA, 2008-11-26 14:00:39 [InstanceEdit:392885] Kanapin, AA, 2009-03-09 12:07:18 [InstanceEdit:400710] Schmidt, EE, 2009-03-25 05:33:35 [InstanceEdit:423310] Kanapin, AA [InstanceEdit:435478] Kanapin, AA [InstanceEdit:435871] Kanapin, AA [InstanceEdit:447347] Kanapin, AA [InstanceEdit:525883] Kanapin, AA [InstanceEdit:613449] Kanapin, AA [InstanceEdit:797602] Kanapin, AA [InstanceEdit:937368] Yung, CK [InstanceEdit:1042053] Yung, CK [InstanceEdit:1220657] Yung, CK [InstanceEdit:1300696] Yung, CK [InstanceEdit:1301627] Yung, CK [InstanceEdit:1551960] Weiser, JD [InstanceEdit:1995863] Weiser, JD [InstanceEdit:2132304] Weiser, JD [InstanceEdit:2265580] Weiser, JD [InstanceEdit:5433710] Weiser, JD [InstanceEdit:5618415] Weiser, JD [InstanceEdit:5634237] Weiser, JD [InstanceEdit:5673015] Weiser, JD [InstanceEdit:9027688] Weiser, JD [InstanceEdit:9037114] Weiser, JD [InstanceEdit:9637257] Weiser, JD [InstanceEdit:9657908] Weiser, JD [InstanceEdit:9676415] Weiser, JD [InstanceEdit:9765872] Weiser, JD [InstanceEdit:9773244] Weiser, Joel [InstanceEdit:9829221] Weiser, Joel [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21 |
| name | Abcd3 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| secondaryIdentifier | ABCD3_MOUSE |
| sequenceLength | 659 |
| species | [Species:48892] Mus musculus |
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No pathways have been reviewed or authored by UniProt:P55096 Abcd3 (84381)
