ABCB7 polyclonal antibody

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. ABCB7 is a half-transporter involved in the transport of heme from the mitochondria to the cytosol and maps to human chromosome Xq13.1-q13.3.