PMP70 Recombinant Rabbit Monoclonal Antibody [JE39-00] (HA721476)

UNIPROT #

P28288

PROTEIN NAME

PMP70

SYNONYMS

70 kDa peroxisomal membrane protein antibody;ABC 43 antibody;ABC D3 antibody;ABC43 antibody;ABCD 3 antibody;Abcd3 antibody;ABCD3 protein antibody;ABCD3_HUMAN antibody;ATP binding cassette sub family D (ALD) member 3 antibody;ATP binding cassette sub family D member 3 antibody;ATP-binding cassette sub-family D member 3 antibody;CBAS5 antibody;dJ824O18.1 (ATP-binding cassette, sub family D (ALD), member 3 (PMP70, PXMP1)) antibody;Peroxisomal membrane protein 1 (70kD) antibody;Peroxisomal membrane protein 1 (70kD, Zellweger syndrome) antibody;Peroxisomal membrane protein 1 antibody;PMP 70 antibody;PMP70 antibody;PXMP 1 antibody;PXMP1 antibody;ZWS2 antibody

SUBCELLULAR LOCATION

Peroxisome membrane.

FUNCTION

ATP-binding cassette sub-family D member 3 is a protein that in humans is encoded by the ABCD3 gene. 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. However, this association was denied [8] and congenital bile acid synthesis defect-5 (CBAS5) was recently shown to be caused by homozygous mutation in the ABCD3 gene.