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MCE 国际站:ABCD1 Protein, Human (His)
ABCD1, Human (His) 是亚家族 D 中四种 ATP 结合盒 (ABC) 蛋白之一。ABCD1 的功能障碍会导致神经退行性疾病。
Synonyms: ABC42; Abcd1; ABCD1_HUMAN; Adrenoleukodystrophy protein; ALD; Aldgh; ALDP; AMN; ATP binding cassette; sub family D ALD; ; member 1; ATP-binding cassette sub-family D member 1; OTTHUMP00000025960; OTTMUSP00000019283; RGD1562128; RP23 373N8.2; X linked adrenoleukodystrophy ALD; gene homolog
Species: Human
Source: E. coli
研究背景:ALDP contains six putative transmembrane domains (TMDs) and a nucleotide-binding domain (NBD). ALDP is synthesized on free polysomes, post-translationally transported to peroxisomes, and inserted into the membranes. During this process, ALDP interacts with Pex19p, a chaperone-like protein for intracellular trafficking of PMP, the complex is targeted to Pex3p on the peroxisomal membranes and then ALDP is inserted into the membranes. After integration into the membranes, ALDP is thought to form mainly a homodimer and in part a heterodimer with other peroxisomal ABC proteins such as 70-kDa peroxisomal membrane protein (PMP70/ABCD3) and adrenoleukodystrophy-related protein (ALDRP/ABCD2). Dysfunction of ABCD1 causes neurodegenerative disorder X-linked adrenoleukodystrophy (X-ALD), which is characterized by the abnormal accumulation of VLCFA due to impaired peroxisomal β-oxidation.
蛋白编号:P33897 (M1-T745)
基因 ID:215
产品分类:其他
Categories:Others
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