Journal of Cell Biology volume 218, issue 1, P206-219 2018 DOI: 10.1083/jcb.201709028 View full text
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Stephen Gilmore-Hall, Jennifer Kuo, Jacqueline M. Ward, Rabaab Zahra, Richard S. Morrison, Guy Perkins, Albert R. La Spada

Abstract: A perplexing question in neurodegeneration is why different neurons degenerate. The Purkinje cell degeneration (pcd) mouse displays a dramatic phenotype of degeneration of cerebellar Purkinje cells. Loss of CCP1/Nna1 deglutamylation of tubulin accounts for pcd neurodegeneration, but the mechanism is unknown. In this study, we modulated the dosage of fission and fusion genes in a Drosophila melanogaster loss-of-function model and found that mitochondrial fragmentation and disease phenotypes were rescued by redu…

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