Article
TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 2 Jun 2010
Ritson Gillian P, Custer Sara K, Freibaum Brian D, Guinto Jake B, Geffel Dyanna, Moore Jennifer, Tang Waixing, Winton Matthew J, Neumann Manuela, Trojanowski John Q, Lee Virginia M-Y, Forman Mark S, Taylor J Paul
Abstract excerpt
Inclusion body myopathy associated with Paget's disease of bone and frontotemporal dementia (IBMPFD) is a dominantly inherited degenerative disorder caused by mutations in the valosin-containing protein (VCP7) gene. VCP (p97 in mouse, TER94 in Drosophila melanogaster, and CDC48 in Saccharomyces cerevisiae) is a highly conserved AAA(+) (ATPases associated with multiple cellular activities) ATPase that regulates a...
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