Anti-ABCD1 antibody (AA29882)

$287.00


Alternative names ATP-binding cassette sub-family D member 1, Adrenoleukodystrophy protein, ALDP, ABCD1, ALD, ABCD1_HUMAN, P33897, 215
Source 745
Species Reactivity Human, Mouse, Rat
Applications IP, IHC, WB
Host Rabbit
Class Polyclonal
Conjugated Unconjugated


Description

Product name Anti-ABCD1 antibody (AA29882)
Catalog number AA29882
Brand family Ango
Product type Primary Antibody
Antibody Alternative Names ATP-binding cassette sub-family D member 1 antibody, Adrenoleukodystrophy protein antibody, ALDP antibody, ABCD1 antibody, ALD antibody, ABCD1_HUMAN antibody


Biological Information

Immunogen Recombinant protein of human ABCD1
Concentration 1 mg/ml
Isotype IgG
Application Notes WB 1:500 - 1:2000 IHC 1:50 - 1:200 IP 1:20 - 1:50
Purification method Purified by affinity chromatography


Target

Target Name ABCD1
SwissProt ID Link P33897
Function Probable transporter. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity.
Subcellular Location Peroxisome membrane; Multi-pass membrane protein.
Involvement in Disease Adrenoleukodystrophy: A peroxisomal metabolic disorder characterized by progressive multifocal demyelination of the central nervous system and by peripheral adrenal insufficiency . It results in mental deterioration, corticospinal tract dysfunction, and cortical blindness. Different clinical manifestations exist like: cerebral childhood ALD , adult cerebral ALD , adrenomyeloneuropathy and 'Addison disease only' phenotype. The disease is caused by mutations affecting the gene represented in this entry. The promoter region of ABCD1 is deleted in the chromosome Xq28 deletion syndrome which involves ABCD1 and the neighboring gene BCAP31.
Subunit Structure Can form homodimers and heterodimers with ABCD2/ALDR and ABCD3/PMP70. Dimerization is necessary to form an active transporter. Interacts with PEX19.
Sequence Similarities Belongs to the ABC transporter superfamily. ABCD family. Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily. Contains 1 ABC transmembrane type-1 domain. Contains 1 ABC transporter domain.


Poduct Presentation

Form Liquid
Preservative 0.02% (w/v) sodium azide
Storage buffer PBS with 50% glycerol, pH 7.3


Storage and Shipping Information

Storage Store at -20 or -80 C. Avoid multiple freeze/thaw cycles.