Article
Inhibition of Pathogenic Mutant SOD1 Aggregation in Cultured Motor Neuronal Cells by Prevention of Its SUMOylation on Lysine 75.
Neuro-degenerative diseases - 1 Jan 2016
Dangoumau Audrey, Marouillat Sylviane, Burlaud Gaillard Julien, Uzbekov Rustem, Veyrat-Durebex Charlotte, Blasco Hélène, Arnoult Christophe, Corcia Philippe, Andres Christian R, Vourc'h Patrick
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the selective death of motor neurons. Mutations in the SOD1 gene encoding the superoxide dismutase 1 are present in 15% of familial ALS cases and in 2% of sporadic cases. These mutations are associated with the formation of SOD1-positive aggregates. The mechanisms of aggregation remain unknown, but posttranslational...
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