BD-PN0479

ABCD3 Rabbit Polyclonal Antibody

常规抗体

ABCD3 PMP70 PXMP1

ATP-binding cassette sub-family D member 3 (70 kDa peroxisomal membrane protein) (PMP70)

WB

Human,Mouse,Rat

1 mg/ml

Liquid in PBS containing 50% glycerol, and 0.02% New type preservative N.

5825

P28288

https://www.uniprot.org/uniprotkb/P28288/entry

P55096

http://www.uniprot.org/uniprot/P55096

P16970

http://www.uniprot.org/uniprot/O54941P16970

Synthesized peptide derived from human protein . at AA range: 40-120

ABCD3 Polyclonal Antibody detects endogenous levels of protein.

WB 1:500-2000 ELISA 1:5000-20000

72kD

-20°C/1 year

Polyclonal, Rabbit,IgG

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 likely plays an important role in peroxisome biogenesis. Mutations have been associated with some forms of Zellweger syndrome, a heterogeneous group of peroxisome assembly disorders. Alternative splicing results in multiple transcript variants

Brain,Liver,Lung,

Peroxisome membrane ; Multi-pass membrane protein .

ABC transporters;

disease:Defects in ABCD3 may be the cause of Zellweger syndrome type 2 (ZWS-2) [MIM:170995]. ZWS-2 is an autosomal recessive disorder due to defective import mechanisms for peroxisomal matrix enzymes. The clinical phenotype includes characteristic facies, progressive neurological dysfunction, liver disease and death in infancy.,function:Probable transporter. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity.,similarity:Belongs to the ABC transporter family. ALD subfamily.,similarity:Contains 1 ABC transmembrane type-1 domain.,similarity:Contains 1 ABC transporter domain.,subunit:Can form heterodimers with ABCD1/ALD and ABCD2/ALDR. Dimerization is necessary to form an active transporter. Interacts with PEX19.,

现货

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