Article
Aberrant folding of pathogenic Parkin mutants: aggregation versus degradation.
The Journal of biological chemistry - 16 May 2008
Schlehe Julia S, Lutz A Kathrin, Pilsl Anna, Lämmermann Kerstin, Grgur Katja, Henn Iris H, Tatzelt Jörg, Winklhofer Konstanze F
Abstract excerpt
Loss-of-function mutations in the Parkin gene (PARK2) are responsible for the majority of autosomal recessive Parkinson disease. A growing body of evidence indicates that misfolding and aggregation of Parkin is a major mechanism of Parkin inactivation, accounting for the loss-of-function phenotype of various pathogenic Parkin mutants. Remarkably, wild-type Parkin is also prone to misfolding under certain cellular...
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