Article
Increased apoptosis, Huntingtin inclusions and altered differentiation in muscle cell cultures from Huntington's disease subjects.
Cell death and differentiation - 1 Dec 2006
Ciammola A, Sassone J, Alberti L, Meola G, Mancinelli E, Russo M A, Squitieri F, Silani V
Abstract excerpt
Mutated huntingtin (htt) is ubiquitously expressed in tissues of Huntington's disease (HD) patients. In the brain, the mutated protein leads to neuronal cell dysfunction and death, associated with formation of htt-positive inclusions. Given increasing evidence of abnormalities in HD skeletal muscle, we extensively analyzed primary muscle cell cultures from seven HD subjects (including two unaffected mutation...
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