Technical Data
031481
ABCD2, CT (ABCD2, ALD1, ALDL1, ALDR, ALDRP, ATP-binding cassette sub-family D member 2, Adrenoleukodystrophy-like 1, Adrenoleukodystrophy-related protein)
Description:
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 Western Blot, Immunohistochemistry, Flow Cytometry and ELISA. Other applications not tested.

Recommended Dilution:
Western Blot: 1:1000
Immunohistochemistry (FFPE): 1:10-1:50
Flow Cytometry: 1:10-1:50
Optimal dilutions to be determined by the researcher.

Storage and Stability:
May be stored at 4C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20C. 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.
TypeIsotypeCloneGrade
PabIgGAffinity Purified
SizeStorageShippingSourceHost
200ul-20CBlue IceHumanRabbit
Concentration:
As Reported
Immunogen:
KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 553-582 in the C-terminal region of human ABCD2, UniProt Accession #Q9UBJ2; NP_005155.1.
Purity:
Purified by Protein A and peptide affinity chromatography.
Form
Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.
Specificity:
Recognizes human ABCD2.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
1. Matsukawa, T., et al. Neurogenetics (2010). 2. Saito, A., et al. J. Hum. Genet. 54(6):317-323 (2009). 3. Maier, E.M., et al. Biochem. Biophys. Res. Commun. 377(1):176-180 (2008). 4. Lu, Y., et al. J. Lipid Res. 49(12):2582-2589 (2008). 5. Petroni, A., et al. J. Inherit. Metab. Dis. 30 (5), 828 (2007)