ABCD2 Rabbit pAb (APR22619N)
产品名称: ABCD2 Rabbit pAb (APR22619N)
英文名称: ABCD2 Rabbit pAb (APR22619N)
产品编号: APR22619N
产品价格: null
产品产地: 美国
品牌商标: Leading Biology
更新时间: null
使用范围: WB (Mus musculus)
安诺伦(北京)生物科技有限公司
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分子量:Calculated MW: 83kDa Observed MW: Refer to figures 细胞定位:Multi-pass membrane protein, Peroxisome membrane 稀释方法:WB 1:500 - 1:2000 IF 1:50 - 1:100 总结: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.