Molecular weight (DA)
75kD
Immunogen
The antiserum was produced against synthesized peptide derived from human ABCD1. AA range:531-580
Specificity
ABCD1 Polyclonal Antibody detects endogenous levels of ABCD1 protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
Western Blot: 1/500 - 1/2000. ELISA: 1/40000. Not yet tested in other applications.
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
The 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
Protein name
ATP-binding cassette sub-family D member 1
Other name
ABCD1; ALD; ATP-binding cassette sub-family D member 1; Adrenoleukodystrophy protein; ALDP
Fields
>>ABC transporters;>>Peroxisome
Human protein sequence Database
P33897
Mouse protein sequence database
P48410
Rat protein sequence database
Cellular localization
Peroxisome membrane ; Multi-pass membrane protein . Mitochondrion membrane ; Multi-pass membrane protein. Lysosome membrane ; Multi-pass membrane protein. Endoplasmic reticulum membrane ; Multi-pass membrane protein.
Tissue expression
Brain,Pancreas,
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