URΦM, Unite de Recherche en Physiologie Moléculaire, University of Namur (FUNDP), Rue de Bruxelles, 61, B-5000 Namur, Belgium.
Curr Drug Metab. 2012 Dec;13(10):1371-87. doi: 10.2174/138920012803762684.
Lysosomes are cytoplasmic organelles delimited by a single membrane and filled with a variety of hydrolytic enzymes active at acidic pH and collectively capable to degrade the vast majority of macromolecules entering lysosomes via endocytosis, phagocytosis or autophagy. In this review, we describe the lipid composition and the dynamic properties of lysosomal membrane, the main delivery pathways of lipids to lysosomes and their catabolism inside lysosomes. Then, we present the consequences of a lipid accumulation as seen in various lysosomal storage diseases on lysosomal functions. Finally, we discuss about the possible involvement of lysosomes in lipotoxicity.
溶酶体是由单层膜界定的细胞质细胞器，内部充满了各种在酸性 pH 值下具有活性的水解酶，这些酶能够共同降解通过内吞作用、吞噬作用或自噬作用进入溶酶体的绝大多数大分子。在这篇综述中，我们描述了溶酶体膜的脂质组成和动态特性、脂质进入溶酶体的主要输送途径及其在溶酶体内的分解代谢。然后，我们介绍了各种溶酶体贮积病中脂质积累对溶酶体功能的影响。最后，我们讨论了溶酶体在脂毒性中的可能作用。
