In vivo metabolic flux profiling with stable isotopes discriminates sites and quantifies effects of mitochondrial dysfunction in C. elegans.

Vergano, Samantha Schrier; Rao, Meera; McCormack, Shana; Ostrovsky, Julian; Clarke, Colleen; Preston, Judith; Bennett, Michael J; Yudkoff, Marc; Xiao, Rui; Falk, Marni J

Vergano, Samantha Schrier; Rao, Meera; McCormack, Shana; Ostrovsky, Julian; Clarke, Colleen; Preston, Judith; Bennett, Michael J; Yudkoff, Marc; Xiao, Rui; Falk, Marni J.

Afiliação

Vergano SS; Division of Human Genetics, The Children's Hospital of Philadelphia, and University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA.
Rao M; Division of Metabolic Disease, The Children's Hospital of Philadelphia and University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA.
McCormack S; Children's Hospital of The King's Daughters, Division of Medical Genetics and Metabolism, Norfolk, VA, USA.
Ostrovsky J; Division of Human Genetics, The Children's Hospital of Philadelphia, and University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA.
Clarke C; Division of Endocrinology, Department of Pediatrics, The Children's Hospital of Philadelphia and University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA.
Preston J; Division of Human Genetics, The Children's Hospital of Philadelphia, and University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA.
Bennett MJ; Division of Human Genetics, The Children's Hospital of Philadelphia, and University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA.
Yudkoff M; Division of Metabolic Disease, The Children's Hospital of Philadelphia and University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA.
Xiao R; Division of Human Genetics, The Children's Hospital of Philadelphia, and University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA.
Falk MJ; Department of Pathology & Laboratory Medicine, The Children's Hospital of Philadelphia and University of Pennsylvania Perelman School of Medicine, Philadelphia, PA 19104, USA.

Mol Genet Metab ; 111(3): 331-341, 2014 Mar.

Article em En | MEDLINE | ID: mdl-24445252

Assuntos

Caenorhabditis elegans/genética; Complexo II de Transporte de Elétrons/genética; Complexo I de Transporte de Elétrons/genética; Mitocôndrias/patologia; Doenças Mitocondriais/genética; Animais; Caenorhabditis elegans/enzimologia; Proteínas de Caenorhabditis elegans/biossíntese; Proteínas de Caenorhabditis elegans/metabolismo; Cromatografia Líquida de Alta Pressão; Complexo I de Transporte de Elétrons/metabolismo; Complexo II de Transporte de Elétrons/metabolismo; Escherichia coli/genética; Humanos; Marcação por Isótopo; Espectrometria de Massas; Redes e Vias Metabólicas; Mitocôndrias/metabolismo; Doenças Mitocondriais/metabolismo; Doenças Mitocondriais/patologia; Mutação

Palavras-chave

High performance liquid chromatography; Mass spectrometry; Pyruvate dehydrogenase deficiency; Respiratory chain mutants; Signaling pathway mutant; TCA cycle mutant

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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Caenorhabditis elegans / Doenças Mitocondriais / Complexo I de Transporte de Elétrons / Complexo II de Transporte de Elétrons / Mitocôndrias Tipo de estudo: Prognostic_studies Limite: Animals / Humans Idioma: En Ano de publicação: 2014 Tipo de documento: Article

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