产品资料

Immunoway/ABCD1 Polyclonal Antibody/50ug 100ug/YT0049

ABCD1 Polyclonal Antibody

Immunogen：
The antiserum was produced against synthesized peptide derived from human ABCD1. AA range:531-580

Dilution：
Western Blot: 1/500 - 1/2000. ELISA: 1/40000. Not yet tested in other applications.

Purification：
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Other Name：
ABCD1; ALD; ATP-binding cassette sub-family D member 1; Adrenoleukodystrophy protein; ALDP

Background：
ATP binding cassette subfamily D member 1(ABCD1)Homo sapiensThe protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the ALD subfamily, which is involved in peroxisomal import of fatty acids and/or fatty acyl-CoAs in the organelle. All known peroxisomal ABC transporters are half transporters which require a partner half transporter molecule to form a functional homodimeric or heterodimeric transporter. This peroxisomal membrane protein is likely involved in the peroxisomal transport or catabolism of very long chain fatty acids. Defects in this gene have been identified as the underlying cause of adrenoleukodystrophy, an X-chromosome recessively inherited demyelinating disord

Function：
disease:Defects in ABCD1 are the cause of adrenoleukodystrophy X-linked (X-ALD) [MIM:300100]. X-ALD is a peroxisomal metabolic disorder characterized by progressive multifocal demyelination of the central nervous system and by peripheral adrenal insufficiency (Addison disease). It results in mental deterioration, corticospinal tract dysfunction, and cortical blindness. Different clinical manifestations exist like: cerebral childhood ALD (CALD), adult cerebral ALD (ACALD), adrenomyeloneuropathy (AMN) and "Addison disease only" (ADO) phenotype.,disease:Microdeletions in ABCD1 are involved in the contiguous ABCD1/DXS1375E deletion syndrome (CADDS) [MIM:300475]. Patients manifest profound neonatal hypotonia, subsequent failure to thrive, and cholestatic liver disease.,function:Probable transporter. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity.,similarity:Be

Subcellular Location：
cytoplasm,mitochondrion,peroxisome,peroxisomal membrane,integral component of peroxisomal membrane,cytosol,membrane,integral component of membrane,perinuclear region of cytoplasm,

Western blot analysis of lysates from Jurkat cells, using ABCD1 Antibody. The lane on the right is blocked with the synthesized peptide.