Neurodegeneration Upon Dysfunction of Endosomal/Lysosomal CLC Chloride Transporters.

ABSTRACT: The regulation of luminal ion concentrations is critical for the function of, and transport between intracellular organelles. The importance of the acidic pH in the compartments of the endosomal-lysosomal pathway has been well-known for decades. Besides the V-ATPase, which pumps protons into their lumen, a variety of ion transporters and channels is involved in the regulation of the organelles' complex ion homeostasis. Amongst these are the intracellular members of the CLC family, ClC-3 through ClC-7. They localize to distinct but overlapping compartments of the endosomal-lysosomal pathway, partially with tissue-specific expression. Functioning as 2Cl^-/H⁺ exchangers, they can support the vesicular acidification and accumulate luminal Cl^-. Mutations in the e

SUBMITTER: Bose S 

PROVIDER: S-EPMC7940362 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature