1-832-868-1888
order@swbio.com
Catalog Number: |
22963 |
other_names: | |
Amount: |
100μg |
calculated_mw: | |
host_species: | Rabbit |
Price: |
$319 |
Swiss-Prot No: |
Swiss-Prot:Q9UBJ2 |
Form of Antibody: |
Supplied in 0.1M Tris-buffered saline with 10% Glycerol (pH7.0). 0.01% Thimerosal was added as a preservative. |
Storage/Stability: |
|
Immunogen: |
Recombinant protein fragment contain a sequence corresponding to a region within amino acids 338 and 558 of Human ABCD2 |
Purification: |
Purified by antigen-affinity chromatography. |
Specificity/Sensitivity: |
|
Applications: |
WB |
Background: |
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. [provided by RefSeq] |
References: |
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appl_detail: |
Predicted MW: 83kd |
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