Altered Reactivity of Superoxide Dismutase in Familial Amyotrophic Lateral Sclerosis

A subset of individuals with familial amyotrophic lateral sclerosis (FALS) possesses dominantly inherited mutations in the gene that encodes copper-zinc superoxide dismutase (CuZnSOD). A4V and G93A, two of the mutant enzymes associated with FALS, were shown to catalyze the oxidation of a model subst...

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Veröffentlicht in:	Science (American Association for the Advancement of Science) 1996-01, Vol.271 (5248), p.515-518
Hauptverfasser:	Wiedau-Pazos, Martina, Goto, Joy J., Rabizadeh, Shahrooz, Gralla, Edith B., Roe, James A., Lee, Michael K., Valentine, Joan S., Bredesen, Dale E.
Format:	Artikel
Sprache:	eng
Schlagworte:	Active sites Adducts Amyotrophic lateral sclerosis Amyotrophic Lateral Sclerosis - enzymology Amyotrophic Lateral Sclerosis - genetics Animals Apoptosis Apoptosis - drug effects Binding Sites Biochemistry Biological and medical sciences Catalysis Cell culture techniques Cell Line Chelating Agents - pharmacology Copper - metabolism Cyclic N-Oxides - metabolism Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Ditiocarb - pharmacology Enzymes Genes Genetic aspects Heredity Humans Hydrogen Peroxide - metabolism Medical sciences Motor neurons Mutation Neurological disorders Neurology Observation Oxidation Oxidation-Reduction Penicillamine - pharmacology Rats Superoxide dismutase Superoxide Dismutase - genetics Superoxide Dismutase - metabolism Yeasts
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