UniprotKB/SwissProt ID: P48410 (P48410)
Gene Name:
Abcd1
Organism: Mus musculus (Mouse)
Function: ATP-dependent transporter of the ATP-binding cassette (ABC) family involved in the transport of very long chain fatty acid (VLCFA)-CoA from the cytosol to the peroxisome lumen. Has fatty acyl-CoA thioesterase (ACOT) and ATPase activities. Coupled to the ATP-dependent transporter activity also has a fatty acyl-CoA thioesterase activity (ACOT) and hydrolyzes VLCFA-CoA into VLCFA prior their ATP-dependent transport into peroxisomes, the ACOT activity is essential during this transport process (By similarity). Thus, plays a role in regulation of VLCFAs and energy metabolism namely, in the degradation and biosynthesis of fatty acids by beta-oxidation, mitochondrial function and microsomal fatty acid elongation (PubMed:18854420, PubMed:23123468, PubMed:23604518, PubMed:25255441, PubMed:25583114, PubMed:26108493, PubMed:9126326, PubMed:9256488, PubMed:9418970). Involved in several processes; namely, controls the active myelination phase by negatively regulating the microsomal fatty acid elongation activity and may also play a role in axon and myelin maintenance (PubMed:11875044, PubMed:15489218, PubMed:26108493). Also controls the cellular response to oxidative stress by regulating mitochondrial functions such as mitochondrial oxidative phosphorylation and depolarization (PubMed:18344354, PubMed:22521832, PubMed:23604518, PubMed:25583114). And finally controls the inflammatory response by positively regulating peroxisomal beta-oxidation of VLCFAs (PubMed:18723473)
Other Modifications: View all modification sites in dbPTM
Protein Subcellular Localization: Peroxisome membrane. Mitochondrion membrane. Lysosome membrane. Endoplasmic reticulum membrane
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