Article
Differences in protein quality control correlate with phenotype variability in 2 mouse models of familial amyotrophic lateral sclerosis.
Neurobiology of aging - 1 Jan 2015
Marino Marianna, Papa Simonetta, Crippa Valeria, Nardo Giovanni, Peviani Marco, Cheroni Cristina, Trolese Maria Chiara, Lauranzano Eliana, Bonetto Valentina, Poletti Angelo, DeBiasi Silvia, Ferraiuolo Laura, Shaw Pamela J, Bendotti Caterina
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a disease of variable severity in terms of speed of progression of the disease course. We found a similar variability in disease onset and progression of 2 familial ALS mouse strains, despite the fact that they carry the same transgene copy number and express the same amount of mutant SOD1G93A messenger RNA and protein in the central nervous system. Comparative analysis of 2...
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