Thermo Fisher Scientific ABCD4 Polyclonal Antibody

Applications

Product Specifications

Product Specific Information

The antibody was affinity-purified from rabbit antiserum by affinity chromatography using an epitope-specific immunogen. The purity is greater than 95% as determined by SDS-PAGE.

Target Information

The peroxisomal membrane contains several ATP-binding cassette (ABC) transporters, ABCD1–4, that are present in the human peroxisome membrane. All four proteins are ABC half-transporters that dimerize to form active transporters.
A mutation in the ABCD1 gene causes X-linked adreno-leukodystrophy (X-ALD), a peroxisomal disorder affecting 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 also comprises two major proteins, PMP22 and ABCD3. ABCD3 is associated with irregularly shaped vesicles that may represent defective peroxisomes or precursors. ABCD1 localizes to peroxisomes, while ABCB7 is a half-transporter involved in heme transport from mitochondria to the cytosol.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.