The peroxisomal membrane contains several ATP-binding cassette (ABC) transporters, ABCD1–4 that are known to be present in the human peroxisome membrane. All four proteins are ABC half-transporters, which dimerize to form an active transporter. A mutation in the ABCD1 causes X-linked adrenoleukodystrophy (X-ALD), a peroxisomal disorder which affects lipid storage. ABCD2 in mouse, is expressed at high levels in the brain and adrenal organs, which are adversely affected in X-ALD. The peroxisomal membrane comprises 2 quantitatively major proteins, PMP22 and ABCD3. ABCD3 is associated with irregularly shaped vesicles which may be defective peroxisomes or peroxisome precursors. ABCD4 localizes to peroxisomes.
应用类型
该ABCD1抗原(D575)产品的应用类型详情查看产品说明书。
免疫原
Synthetic peptide, corresponding to amino acids 550-600 of Human ABCD1.