Article
Differential substrate specificities of human ABCD1 and ABCD2 in peroxisomal fatty acid β-oxidation.
Biochimica et biophysica acta - 1 Mar 2011
van Roermund Carlo W T, Visser Wouter F, Ijlst Lodewijk, Waterham Hans R, Wanders Ronald J A
Abstract excerpt
The gene mutated in X-linked adrenoleukodystrophy (X-ALD) codes for the HsABCD1 protein, also named ALDP, which is a member of the superfamily of ATP-binding cassette (ABC) transporters and required for fatty acid transport across the peroxisomal membrane. Although a defective HsABCD1 results in the accumulation of very long-chain fatty acids in plasma of X-ALD patients, there is still no direct biochemical...
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