作者： Robert Hegele , Daniele Pastori , Hanrui Zhang , Yuan Xinxu , Gordon A Francis
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摘要: Lysosomal acid lipase (LAL), encoded by the gene LIPA, is the sole lysosomal lipase, responsible for the critical functions of hydrolysis of cholesteryl esters (CE) contained in endocytosed lipoproteins to component free cholesterol and fatty acids, and of triglycerides to their component fatty acids. LAL also cleaves stored CE delivered to lysosomes for breakdown in the lipophagy component of autophagy (Ouimet et al., 2011), enhancement of which may be protective against atherosclerosis (Sergin et al., 2017). Complete deficiency of LAL, Wolman Disease (WD), is fatal in early life due to