X-连锁肾上腺-脑白质营养不良蛋白的结构与功能

X-连锁肾上腺 脑白质营养不良基因（ALD基因）编码的ALD蛋白（ALDP）是4种人类ABCD转运蛋白之一，为一种半ABC转运蛋白，既有ABC(ATP binding cassette)转运蛋白的共有特征，又有过氧化物酶体膜蛋白的特点. 其功能可能是将胞浆中极长链饱和脂肪酸（VLCFA）或其衍生物转运到过氧化物酶体内，并在其中进行β氧化. 已报道的ALD基因突变有900多个，其后果多种多样，但最终都使VLCFA或其衍生物无法进入过氧化物酶体，从而使VLCFA在体内蓄积. 作者认为，ALDP是研究ABCD转运蛋白，乃至所有ABC转运蛋白的一个极好模型.

Structure and Function of X-linked Adrenoleukodystrophy Protein

ALD protein (ALDP), encoded by X-linked adrenoleukodystrophy gene (ALD gene), is one of the four ABCD transporters in human being. As a half ABC (ATP binding cassette) transporter, it possesses the properties shared by all ABC transporters, while it is characteristic of peroxisomal proteins. It functions probably in transporting very long chain fatty acids (VLCFA) or its derivatives from the cytosol into the interior of peroxisome to undergo β-oxidation. More than 900 mutations have been reported, which lead to multiple sequels, with the end result being that the VLCFA or its derivatives is unable to enter the peroxisome and accumulated in the body. We believe that ALDP is an excellent research model for the study of ABCD transporters, or even ABC transporters as a whole.