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Article
Biochemistry & Molecular Biology
Parkinson's disease-risk protein TMEM175 is a proton-activated proton channel in lysosomes
Meiqin Hu et al.
Summary: A genetic risk factor for Parkinson's disease, TMEM175, acts as a proton-activated, proton-selective channel on the lysosomal membrane to regulate lysosomal H+ leak. Activation of TMEM175 stops further acidification of lysosomes beyond the normal range. Deficiency of TMEM175 causes lysosomal over-acidification, impaired proteolytic activity, and facilitates alpha-synuclein aggregation.
CELL (2022)