Abstract：
Lysosomes degrade and recycle macromolecules that are delivered through the biosynthetic, endocytic, and autophagic routes. Hydrolysis of the different classes of macromolecules is catalyzed by about 70 soluble enzymes that are transported from the Golgi apparatus to lysosomes in a mannose 6-phosphate (M6P)-dependent process. The molecular machinery that generates M6P tags for receptor-mediated targeting of lysosomal enzymes was thought to be understood in detail. However, recent studies on the M6P pathway have identified a previously uncharacterized core component, yielded structural insights in known components, and uncovered functions in various human diseases. Here we review molecular mechanisms of lysosomal enzyme trafficking and discuss its relevance for rare lysosomal disorders, cancer, and viral infection.
摘要：
溶酶体降解和回收通过生物合成传递的大分子，内吞,和自噬路线。不同类型的大分子的水解由约70种可溶性酶催化，这些酶在甘露糖6-磷酸（M6P）依赖性过程中从高尔基体转运到溶酶体。产生用于受体介导的溶酶体酶靶向的M6P标签的分子机制被认为是详细理解的。然而,最近对M6P途径的研究已经确定了一个以前未表征的核心成分，在已知组件中产生了结构见解，并揭示了各种人类疾病的功能。在这里，我们回顾了溶酶体酶运输的分子机制，并讨论了其与罕见溶酶体疾病的相关性。癌症,和病毒感染。
