Combinatorial glucose, nicotinic acid and N-acetylcysteine therapy has synergistic effect in preclinical C. elegans and zebrafish models of mitochondrial complex I disease.

Guha, Sujay; Mathew, Neal D; Konkwo, Chigoziri; Ostrovsky, Julian; Kwon, Young Joon; Polyak, Erzsebet; Seiler, Christoph; Bennett, Michael; Xiao, Rui; Zhang, Zhe; Nakamaru-Ogiso, Eiko; Falk, Marni J

Guha, Sujay; Mathew, Neal D; Konkwo, Chigoziri; Ostrovsky, Julian; Kwon, Young Joon; Polyak, Erzsebet; Seiler, Christoph; Bennett, Michael; Xiao, Rui; Zhang, Zhe; Nakamaru-Ogiso, Eiko; Falk, Marni J.

Afiliação

Guha S; Mitochondrial Medicine Frontier Program, Division of Human Genetics, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Mathew ND; Mitochondrial Medicine Frontier Program, Division of Human Genetics, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Konkwo C; Mitochondrial Medicine Frontier Program, Division of Human Genetics, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Ostrovsky J; Mitochondrial Medicine Frontier Program, Division of Human Genetics, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Kwon YJ; Mitochondrial Medicine Frontier Program, Division of Human Genetics, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Polyak E; Mitochondrial Medicine Frontier Program, Division of Human Genetics, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Seiler C; Aquatics Core Facility, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Bennett M; Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia and University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.
Xiao R; Department of Biostatistics, Epidemiology and Informatics, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.
Zhang Z; Center for Biomedical Informatics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Nakamaru-Ogiso E; Mitochondrial Medicine Frontier Program, Division of Human Genetics, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Falk MJ; Mitochondrial Medicine Frontier Program, Division of Human Genetics, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Hum Mol Genet ; 30(7): 536-551, 2021 05 12.

Article em En | MEDLINE | ID: mdl-33640978

Assuntos

Acetilcisteína/farmacologia; Modelos Animais de Doenças; Complexo I de Transporte de Elétrons/metabolismo; Glucose/farmacologia; Mitocôndrias/efeitos dos fármacos; Doenças Mitocondriais/prevenção & controle; Niacina/farmacologia; Animais; Caenorhabditis elegans; Sinergismo Farmacológico; Complexo I de Transporte de Elétrons/genética; Sequestradores de Radicais Livres/farmacologia; Humanos; Longevidade/efeitos dos fármacos; Longevidade/genética; Potencial da Membrana Mitocondrial/efeitos dos fármacos; Mitocôndrias/genética; Mitocôndrias/metabolismo; Doenças Mitocondriais/genética; Doenças Mitocondriais/metabolismo; Mutação; Estresse Oxidativo/efeitos dos fármacos; Peixe-Zebra

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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Acetilcisteína / Doenças Mitocondriais / Complexo I de Transporte de Elétrons / Modelos Animais de Doenças / Glucose / Mitocôndrias / Niacina Tipo de estudo: Clinical_trials / Prognostic_studies Limite: Animals / Humans Idioma: En Ano de publicação: 2021 Tipo de documento: Article

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