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NSJ Bio/ABCD2 Antibody (F42334)/0.08 ml/F42334-0.08ML

Description

The protein encoded by this gene is a member of thesuperfamily of ATP-binding cassette (ABC) transporters. ABCproteins transport various molecules across extra- andintra-cellular membranes. ABC genes are divided into seven distinctsubfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). Thisprotein is a member of the ALD subfamily, which is involved inperoxisomal import of fatty acids and/or fatty acyl-CoAs in theorganelle. All known peroxisomal ABC transporters are halftransporters which require a partner half transporter molecule toform a functional homodimeric or heterodimeric transporter. Thefunction of this peroxisomal membrane protein is unknown; howeverthis protein is speculated to function as a dimerization partner ofABCD1 and/or other peroxisomal ABC transporters. Mutations in thisgene have been observed in patients with adrenoleukodystrophy, asevere demyelinating disease. This gene has been identified as acandidate for a modifier gene, accounting for the extreme variationamong adrenoleukodystrophy phenotypes. This gene is also acandidate for a complement group of Zellweger syndrome, agenetically heterogeneous disorder of peroxisomal biogenesis.

Application Notes

Titration of the ABCD2 antibody may be required due to differences in protocols and secondary/substrate sensitivity.

Immunogen

A portion of amino acids 553-582 from the human protein was used as the immunogen for this ABCD2 antibody.

Storage

Aliquot the ABCD2 antibody and store frozen at -20oC or colder.Avoid repeated freeze-thaw cycles.

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