Glucocerebrosidase mutations disrupt the lysosome and now the mitochondria.

Klein, Andrés D; Outeiro, Tiago Fleming · Nat Commun · 2023

basic_science · Level V

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Abstract

β-Glucocerebrosidase (GCase) mutations lead to glucosylceramide build-up in the lysosome, impacting α-synuclein aggregation and autophagy. Recently, Baden and colleagues found GCase in mitochondria, supporting mitochondrial complex I function and energy metabolism. We believe the newly described role of GCase in the mitochondria will inform new Parkinson’s and Gaucher’s disease therapeutics.

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