Pathogenic VCP/TER94 Alleles Are Dominant Actives and Contribute to Neurodegeneration by Altering Cellular ATP Level in a Drosophila IBMPFD Model

Inclusion body myopathy with Paget's disease of bone and frontotemporal dementia (IBMPFD) is caused by mutations in Valosin-containing protein (VCP), a hexameric AAA ATPase that participates in a variety of cellular processes such as protein degradation, organelle biogenesis, and cell-cycle reg...

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Detalles Bibliográficos
Autores principales: Chang, Ya-Chu, Hung, Wan-Tzu, Chang, Yun-Chin, Chang, Henry C., Wu, Chia-Lin, Chiang, Ann-Shyn, Jackson, George R., Sang, Tzu-Kang
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2011
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3033380/
https://www.ncbi.nlm.nih.gov/pubmed/21304887
http://dx.doi.org/10.1371/journal.pgen.1001288

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