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溶酶体贮积症:溶酶体内部及以外的病理学。

Lysosomal storage disorders: pathology within the lysosome and beyond.

机构信息

Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, Nebraska, USA.

出版信息

J Neurochem. 2019 Mar;148(5):568-572. doi: 10.1111/jnc.14672.

DOI:10.1111/jnc.14672
PMID:30697734
Abstract

This Preface introduces the articles of the special issue on "Lysosomal Storage Disorders" in which several recognized experts provide an overview of this research field. Lysosomes were first described in the 1950s and recognized for their role in substrate degradation and recycling. Because lysosomes impact numerous fundamental homeostatic processes, research on lysosomal storage disorders (LSDs) is crucial to advance our understanding of this intriguing organelle. This Special Issue highlights some of the LSDs that impact the central nervous system (CNS) as well as comprehensive overviews of lysosomal biology, CNS metabolism, and sphingolipid biosynthesis and turnover, all of which are critical toward our understanding of normal lysosomal function and how this is perturbed in the context of LSDs. This is the Preface for the special issue "Lysosomal Storage Disorders". Cover Image for this issue: doi: 10.1111/jnc.14496.

摘要

这篇序言介绍了特刊中关于“溶酶体贮积症”的文章,几位知名专家对这一研究领域进行了概述。溶酶体于 20 世纪 50 年代首次被描述,并因其在底物降解和再循环中的作用而被认识。由于溶酶体影响许多基本的动态平衡过程,因此研究溶酶体贮积症(LSDs)对于深入了解这一有趣的细胞器至关重要。本特刊重点介绍了一些影响中枢神经系统(CNS)的溶酶体贮积症,以及对溶酶体生物学、CNS 代谢和鞘脂生物合成和周转的全面概述,所有这些对于理解正常溶酶体功能以及 LSD 背景下溶酶体功能如何受到干扰都至关重要。这是“溶酶体贮积症”特刊的序言。本期特刊的封面图片:doi: 10.1111/jnc.14496.

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