Centro de Genética y Genómica, Facultad de Medicina, Clínica Alemana Universidad del Desarrollo, Santiago, 7780272, Chile.
Department of Experimental Neurodegeneration, Center for Biostructural Imaging of Neurodegeneration, University Medical Center Göttingen, Göttingen, Germany.
Nat Commun. 2023 Oct 11;14(1):6383. doi: 10.1038/s41467-023-42107-7.
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.
β-葡萄糖脑苷脂酶 (GCase) 突变导致溶酶体中葡萄糖神经酰胺的积累，影响α-突触核蛋白的聚集和自噬。最近，Baden 及其同事在线粒体中发现了 GCase，支持线粒体复合物 I 的功能和能量代谢。我们相信 GCase 在新描述的线粒体中的作用将为帕金森病和戈谢病的新疗法提供信息。
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