Article
Progressive impairment of CaV1.1 function in the skeletal muscle of mice expressing a mutant type 1 Cu/Zn superoxide dismutase (G93A) linked to amyotrophic lateral sclerosis.
Skeletal muscle - 1 Jan 2016
Beqollari Donald, Romberg Christin F, Dobrowolny Gabriella, Martini Martina, Voss Andrew A, Musarò Antonio, Bannister Roger A
Abstract excerpt
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disorder that is typically fatal within 3-5 years of diagnosis. While motoneuron death is the defining characteristic of ALS, the events that underlie its pathology are not restricted to the nervous system. In this regard, ALS muscle atrophies and weakens significantly before presentation of neurological symptoms. Since the...
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