Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
10882948 | Mitochondrion | 2012 | 10 Pages |
Abstract
⺠Pdss2 deficient mice exhibit severe neuromuscular defects as demonstrated by behavioral testing. ⺠Marked reduction in TH-positive cells in the substantia nigra reflects neuromuscular impairment. ⺠Probucol prevented kidney disease, but not neuromuscular phenotypes, in Pdss2 missense mutants.
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Authors
Carly G.K. Ziegler, Min Peng, Marni J. Falk, Erzsebet Polyak, Elpida Tsika, Harry Ischiropoulos, Dana Bakalar, Julie A. Blendy, David L. Gasser,