ABCD2, CT (ABCD2, ALD1, ALDL1, ALDR, ALDRP, ATP-binding cassette sub-family D member 2, Adrenoleukodystrophy-like 1, Adrenoleukodystrophy-related protein) (PE)

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.\n\nApplications:\nSuitable for use in Western Blot, Immunohistochemistry, Flow Cytometry, ELISA\n\nRecommended Dilution:\nELISA: 1:1,000\nWestern Blot: 1:100-500\nImmunohistochemistry: 1:10-50\nFlow Cytometry: 1:10-50\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Do not freeze R-Phycoerythrin conjugates. Light sensitive. Aliquots are stable for 6 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

031481-PE

Size

200ul

Applications

ELISA, FC, IHC, WB

Hosts

Rabbit

Reactivities

Hum

Form

Supplied as a liquid in PBS, pH 7.2. Contains ~0.09% sodium azide. Conjugated to R-Phycoerythrin (R-PE).

P Type

Pab

Purity

Purified by Protein A affinity chromatography.

Isotype

IgG

References

Matsukawa, T., et al. Neurogenetics (2010) In press :\nSaito, A., et al. J. Hum. Genet. 54(6):317-323(2009)\nMaier, E.M., et al. Biochem. Biophys. Res. Commun. 377(1):176-180(2008)\nLu, Y., et al. J. Lipid Res. 49(12):2582-2589(2008)\nPetroni, A., et al. J. Inherit. Metab. Dis. 30 (5), 828 (2007) :

Additional Info

Human

Alternative Names

ABCD2, ALD1, ALDL1, ALDR, ALDRP, ATP-binding cassette sub-family D member 2, Adrenoleukodystrophy-like 1, Adrenoleukodystrophy-related protein