| Lysosomal cholesterol accumulation is commonly found in most peroxisomal disorders and reversed by 2-hydroxypropyl-β-cyclodextrin |
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| 作者姓名： | Lewei Dong Jian Xiao Shuai Liu Gang Deng Yacheng Liao Beibei Chu Xiaolu Zhao Bao-Liang Song Jie Luo |
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| 作者单位： | 1. College of Life Sciences, Taikang Center for Life and Medical Sciences, Hubei Key Laboratory of Cell Homeostasis, Wuhan University;2. College of Veterinary Medicine, Henan Agricultural University |
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| 基金项目： | supported by the China Postdoctoral Science Foundation Grant (2021M692478)；;the National Natural Science Foundation of China (32293203, 31771568)；;111 Project of Ministry of Education of China (B16036)； |
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| 摘 要： | Peroxisomal disorders(PDs) are a heterogenous group of diseases caused by defects in peroxisome biogenesis or functions. Xlinked adrenoleukodystrophy is the most prevalent form of PDs and results from mutations in the ABCD1 gene, which encodes a transporter mediating the uptake of very long-chain fatty acids(VLCFAs). The curative approaches for PDs are very limited.Here, we investigated whether cholesterol accumulation in the lysosomes is a biochemical feature shared by a broad spectrum of PDs. ...
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