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

@inproceedings{Chang2011PathogenicVA,
  title={Pathogenic VCP/TER94 Alleles Are Dominant Actives and Contribute to Neurodegeneration by Altering Cellular ATP Level in a Drosophila IBMPFD Model},
  author={Ya-chu Chang and Wan-Tzu Hung and Yun-Chin Chang and Henry C. Chang and Chia-Lin Wu and A. M. Chiang and George R. Jackson and Tzu-kang Sang},
  booktitle={PLoS genetics},
  year={2011}
}
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 (the sole Drosophila VCP ortholog) carrying mutations analogous to those implicated in… CONTINUE READING
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