UniProt ID: F1RBC8 |
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. Coupled to the ATP- dependent transporter activity has also 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. 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 (By similarity). Involved in oligodendrocyte patterning and myelination (PubMed:28911205). {ECO:0000250|UniProtKB:P33897, ECO:0000269|PubMed:28911205}. CATALYTIC ACTIVITY: Reaction=a very long-chain fatty acyl-CoA + H2O = a very long-chain fatty acid + CoA + H(+); Xref=Rhea:RHEA:67072, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:57287, ChEBI:CHEBI:58950, ChEBI:CHEBI:138261; Evidence={ECO:0000250|UniProtKB:P33897}; CATALYTIC ACTIVITY: Reaction=a very long-chain fatty acid(in) + ATP + H2O = a very long- chain fatty acid(out) + ADP + H(+) + phosphate; Xref=Rhea:RHEA:67080, ChEBI:CHEBI:15377, ChEBI:CHEBI:15378, ChEBI:CHEBI:30616, ChEBI:CHEBI:43474, ChEBI:CHEBI:58950, ChEBI:CHEBI:456216; Evidence={ECO:0000250|UniProtKB:P33897}; SUBCELLULAR LOCATION: Peroxisome membrane {ECO:0000250|UniProtKB:P33897}; Multi-pass membrane protein {ECO:0000255}. DEVELOPMENTAL STAGE: Expressed from 48 hpf in the nervous system, floor plate, and pronephros/interrenal gland. By 72 hpf and persisting through 96 hpf expression is present in the central nervous system (CNS), floorplate, interrenal glands/pronephros, and gut. At 72 hpf expressed in the brain and spinal cord. {ECO:0000269|PubMed:28911205}. DISRUPTION PHENOTYPE: abcd1 deficient zebrafish appear normal. By day of life five abcd1 mutants demonstrate impaired motor function, and overall survival to adulthood of heterozygous and homozygous mutants is decreased. {ECO:0000269|PubMed:28911205}. SIMILARITY: Belongs to the ABC transporter superfamily. ABCD family. Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily. {ECO:0000305}. |
This information was provided by UniProt through a collaboration with ZFIN. (1) |