Cyclohexane 1, 3-diones and their inhibition of mutant SOD1-dependent protein aggregation and toxicity in PC12 cells

…, R Benmohamed, AC Arvanites, RI Morimoto…

Index: Zhang, Wei; Benmohamed, Radhia; Arvanites, Anthony C.; Morimoto, Richard I.; Ferrante, Robert J.; Kirsch, Donald R.; Silverman, Richard B. Bioorganic and Medicinal Chemistry, 2012 , vol. 20, # 2 p. 1029 - 1045

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Citation Number: 10

Abstract

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the progressive loss of motor neurons. Currently, there is only one FDA-approved treatment for ALS (riluzole), and that drug only extends life, on average, by 2–3months. Mutations in Cu/Zn superoxide dismutase (SOD1) are found in familial forms of the disease and have played an important role in the study of ALS pathophysiology. On the basis of their activity ...