The molecular tweezer CLR01 inhibits aberrant superoxide dismutase 1 (SOD1) self-assembly <i>in vitro</i> and in the G93A-SOD1 mouse model of ALS.

Malik, Ravinder; Meng, Helen; Wongkongkathep, Piriya; Corrales, Christian I; Sepanj, Niki; Atlasi, Ryan S; Klärner, Frank-Gerrit; Schrader, Thomas; Spencer, Melissa J; Loo, Joseph A; Wiedau, Martina; Bitan, Gal

The molecular tweezer CLR01 inhibits aberrant superoxide dismutase 1 (SOD1) self-assembly in vitro and in the G93A-SOD1 mouse model of ALS.

Malik, Ravinder; Meng, Helen; Wongkongkathep, Piriya; Corrales, Christian I; Sepanj, Niki; Atlasi, Ryan S; Klärner, Frank-Gerrit; Schrader, Thomas; Spencer, Melissa J; Loo, Joseph A; Wiedau, Martina; Bitan, Gal.

Afiliación

Malik R; From the Department of Neurology, David Geffen School of Medicine, and.
Meng H; From the Department of Neurology, David Geffen School of Medicine, and.
Wongkongkathep P; Departments of Chemistry and Biochemistry and.
Corrales CI; From the Department of Neurology, David Geffen School of Medicine, and.
Sepanj N; From the Department of Neurology, David Geffen School of Medicine, and.
Atlasi RS; From the Department of Neurology, David Geffen School of Medicine, and.
Klärner FG; the Faculty of Chemistry, University of Duisburg-Essen, 47057 Essen, Germany.
Schrader T; the Faculty of Chemistry, University of Duisburg-Essen, 47057 Essen, Germany.
Spencer MJ; From the Department of Neurology, David Geffen School of Medicine, and.
Loo JA; Brain Research Institute, and.
Wiedau M; Departments of Chemistry and Biochemistry and.
Bitan G; Biological Chemistry.

J Biol Chem ; 294(10): 3501-3513, 2019 03 08.

Article en En | MEDLINE | ID: mdl-30602569

Asunto(s)

Esclerosis Amiotrófica Lateral/metabolismo; Hidrocarburos Aromáticos con Puentes/farmacología; Mutación; Organofosfatos/farmacología; Superóxido Dismutasa-1/química; Superóxido Dismutasa-1/genética; Secuencia de Aminoácidos; Esclerosis Amiotrófica Lateral/genética; Esclerosis Amiotrófica Lateral/fisiopatología; Animales; Sitios de Unión; Peso Corporal/efectos de los fármacos; Hidrocarburos Aromáticos con Puentes/metabolismo; Modelos Animales de Enfermedad; Ratones; Fuerza Muscular/efectos de los fármacos; Organofosfatos/metabolismo; Agregado de Proteínas/efectos de los fármacos; Médula Espinal/efectos de los fármacos; Médula Espinal/metabolismo; Superóxido Dismutasa-1/metabolismo; Análisis de Supervivencia

Palabras clave

amyloid; amyotrophic lateral sclerosis (ALS) (Lou Gehrig disease); inhibitor; molecular tweezer; motor neuron; mouse; neurodegeneration; protein aggregation; protein misfolding; superoxide dismutase (SOD)

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Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Organofosfatos / Hidrocarburos Aromáticos con Puentes / Superóxido Dismutasa-1 / Esclerosis Amiotrófica Lateral / Mutación Tipo de estudio: Prognostic_studies Límite: Animals Idioma: En Revista: J Biol Chem Año: 2019 Tipo del documento: Article

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