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Science China Life Sciences

, Volume 59, Issue 8, pp 777–791

First Online: 19 July 2016Received: 06 May 2016Accepted: 02 June 2016DOI: 10.1007-s11427-016-5090-x

Cite this article as: Xiong, J. & Zhu, M.X. Sci. China Life Sci. 2016 59: 777. doi:10.1007-s11427-016-5090-x

Abstract

Lysosomes are the major organelles that carry out degradation functions. They integrate and digest materials compartmentalized by endocytosis, phagocytosis or autophagy. In addition to more than 60 hydrolases residing in the lysosomes, there are also ion channels and transporters that mediate the flux or transport of H, Ca, Na, K, and Cl across the lysosomal membranes. Defects in ionic exchange can lead to abnormal lysosome morphology, defective vesicle trafficking, impaired autophagy, and diseases such as neurodegeneration and lysosomal storage disorders. The latter are characterized by incomplete lysosomal digestion and accumulation of toxic materials inside enlarged intracellular vacuoles. In addition to degradation, recent studies have revealed the roles of lysosomes in metabolic pathways through kinases such as mechanistic target of rapamycin mTOR and transcriptional regulation through calcium signaling molecules such as transcription factor EB TFEB and calcineurin. Owing to the development of new approaches including genetically encoded fluorescence probes and whole endolysosomal patch clamp recording techniques, studies on lysosomal ion channels have made remarkable progress in recent years. In this review, we will focus on the current knowledge of lysosome-resident ion channels and transporters, discuss their roles in maintaining lysosomal function, and evaluate how their dysfunction can result in disease.

Keywordslysosomal storage disease LSD ion homeostasis calcium lysosome acidification This article is published with open access at link.springer.com

Open Access This article is distributed under the terms of the Creative Commons Attribution License which permits any use, distribution, and reproduction in any medium, provided the original authors and source are credited.

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Author: Jian Xiong - Michael X. Zhu

Source: https://link.springer.com/



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