Article
SVIP is a molecular determinant of lysosomal dynamic stability, neurodegeneration and lifespan.
Nature communications - 21 Jan 2021
Johnson Alyssa E, Orr Brian O, Fetter Richard D, Moughamian Armen J, Primeaux Logan A, Geier Ethan G, Yokoyama Jennifer S, Miller Bruce L, Davis Graeme W
Abstract excerpt
Missense mutations in Valosin-Containing Protein (VCP) are linked to diverse degenerative diseases including IBMPFD, amyotrophic lateral sclerosis (ALS), muscular dystrophy and Parkinson's disease. Here, we characterize a VCP-binding co-factor (SVIP) that specifically recruits VCP to lysosomes. SVIP is essential for lysosomal dynamic stability and autophagosomal-lysosomal fusion. SVIP mutations cause muscle...
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