A Lysosomal Lair for a Pathogenic Protein Pair

作者：Dawson Ted M^*; Dawson Valina L

来源：Science Translational Medicine, 2011, 3(91): 91ps28.

DOI：10.1126/scitranslmed.3002808

Parkinson's disease (PD) is a progressive neurodegenerative disorder that affects movement. Although many of the causes of PD remain unclear, a consistent finding is the abnormal accumulation of the protein alpha-synuclein. In a recent issue of Cell, Mazzuli et al. provide a molecular explanation for the unexpected link between PD and Gaucher's disease, a glycolipid lysosomal storage disorder caused by loss of the enzyme glucocerebrosidase (GBA). They report a reciprocal connection between loss of GBA activity and the accumulation of alpha-synuclein in lysosomes that establishes a bidirectional positive feedback loop with pathogenic consequences. Understanding how lysosomes are implicated in PD may reveal new therapeutic targets for treating this disease.

• 出版日期2011-7-13