ATP-binding cassette sub-family D member 1 (ABCD1)

Plays a role in the transport of free very-long-chain fatty acids (VLCFAs) in addition to their CoA-esters across the peroxisomal membrane by acting as an ATP-specific binding subunit releasing ADP following ATP hydrolysis (PubMed:15682271, PubMed:11248239, PubMed:16946495). 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:23671276).

Involved in many procedures; namely, controls the active myelination stage by negatively regulating the microsomal fatty acid elongation activity and could also play a role in axon and myelin maintenance. Controls also the cellular response to oxidative stress by regulating adrenal function such as, mitochondrial oxidative phosphorylation and depolarization.