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167339

ABCD2 (ABC39, Adrenoleukodystrophy-like 1, Adrenoleukodystrophy-related protein, ALDL1, ALDR, ALDRP, ATP-binding cassette sub-family D member 2, ATP-binding cassette, Sub-family D (ALD), Member 2, hALDR)

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£642.00

SKU:
167339
Reinheit:
Purified by ammonium sulfate precipitation.
Lagerbedingungen:
-20°C
Hersteller:
United States Biological
Host:
Goat
Immunogen:
Peptide with sequence C-PLSDTLAIKGKVID, from the internal region of the protein. (NP_005155.1).
Format:
Supplied as a liquid in Tris saline, pH 7.3, 0.5% BSA, 0.02% sodium azide.
Weitere Details:
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. The function of this peroxisomal membrane protein is unknown; however this protein is speculated to function as a dimerization partner of ABCD1 and/or other peroxisomal ABC transporters. Mutations in this gene have been observed in patients with adrenoleukodystrophy, a severe demyelinating disease. This gene has been identified as a candidate for a modifier gene, accounting for the extreme variation among adrenoleukodystrophy phenotypes. This gene is also a candidate for a complement group of Zellweger syndrome, a genetically heterogeneous disorder of peroxisomal biogenesis. Applications: Suitable for use in ELISA and Western Blot. Other applications not tested. Recommended Dilution: ELISA: 1:32,000 Western Blot: 0.5-2ug/ml Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Versandbedingungen:
Blue Ice