Article
Pathogenic VCP/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD model.
PLoS genetics - 3 Feb 2011
Chang Ya-Chu, Hung Wan-Tzu, Chang Yun-Chin, Chang Henry C, Wu Chia-Lin, Chiang Ann-Shyn, Jackson George R, Sang Tzu-Kang
Abstract excerpt
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 regulation. To understand how VCP mutations cause IBMPFD, we have established a Drosophila model by overexpressing TER94...
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