|Other Names||ATP-binding cassette sub-family D member 4, PMP70-related protein, P70R, Peroxisomal membrane protein 1-like, PXMP1-L, Peroxisomal membrane protein 69, PMP69, ABCD4, PXMP1L|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP9267c was selected from the Center region of human ABCD4. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Lysosomal transporter that plays a role in the lysosomal release of vitamin B12 into the cytosol (PubMed:22922874). Targeted by LMBRD1 lysosomal chaperone from the endoplasmic reticulum to the lysosomal membrane (PubMed:27456980). Then forms a complex with lysosomal chaperone LMBRD1 and cytosolic MMACHC to transport cobalamin across the lysosomal membrane (PubMed:25535791).|
|Cellular Location||Endoplasmic reticulum membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein|
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Provided below are standard protocols that you may find useful for product applications.
ABCD4 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. The function of this peroxisomal membrane protein is unknown. However, it is speculated that it may function as a heterodimer for another peroxisomal ABC transporter and, therefore, may modify the adrenoleukodystrophy phenotype. It may also play a role in the process of peroxisome biogenesis.
Saito,A., et.al, J. Hum. Genet. 54 (6), 317-323 (2009)Asheuer,M., et.al. Hum. Mol. Genet. 14 (10), 1293-1303 (2005)Kuiper,H., et.al, Cytogenet. Genome Res. 109 (4), 533 (2005)
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