ATP-binding cassette sub-family D member 2 (ABCD2)

ATP-binding cassette sub-family D member 2 (ABCD2) is a peroxisomal membrane transporter protein and a member of the ATP-binding cassette (ABC) transporter superfamily[6][7]. It is a ‘half-transporter’ that forms functional homodimers (or in some contexts, heterodimers) to mediate the ATP-dependent import of specific long-chain and very long–chain fatty acyl-CoA substrates from the cytosol into the peroxisome, where these molecules are degraded through β-oxidation[2][5][8]. ABCD2 has a substrate profile that partially overlaps with, but is distinct from, its close homolog ABCD1; it is particularly effective for C22:0 and certain polyunsaturated fatty acids[2][5]. Mutations or dysregulation of ABCD2 have been implicated in severe peroxisomal disorders, notably adrenoleukodystrophy and Zellweger syndrome, where it may act as a modifier of disease severity[5][6]. Currently, no drugs directly target ABCD2, but it carries importance as a possible therapeutic target or modifier in treating peroxisomal lipid metabolism disorders[6][7].

Other names

ATP-binding cassette sub-family D member 2ABCD2Adrenoleukodystrophy-related proteinALDRALD1ALDL1ALDRPhALDRABC39Adrenoleukodystrophy-like 1

Facilitates ATP-dependent transport of fatty acyl-CoAs into peroxisomes for β-oxidation; hypothesized fatty acyl-CoA thioesterase and ATPase activities[5][7]