Phenylbutyrate Therapy for Pyruvate Dehydrogenase Complex Deficiency and Lactic Acidosis

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  Phenylbutyrate Therapy for Pyruvate Dehydrogenase Complex Deficiency and Lactic Acidosis

  By Rosa Ferriero, Giuseppe Manco, Eleonora Lamantea, Edoardo Nusco, Maria I. Ferrante, Paolo Sordino, Peter W. Stacpoole, Brendan Lee, Massimo Zeviani, Nicola Brunetti-Pierri

  Science Translational Medicine06 Mar 2013 : 175ra31

  Phenylbutyrate enhances pyruvate dehydrogenase enzyme complex (PDHC) activity through inhibition of PDHC kinase, rescues the severe phenotype of a zebrafish model of PDHC deficiency, and prevents systemic lactic acidosis.

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  Phenylbutyrate Therapy for Pyruvate Dehydrogenase Complex Deficiency and Lactic Acidosis

  By Rosa Ferriero, Giuseppe Manco, Eleonora Lamantea, Edoardo Nusco, Maria I. Ferrante, Paolo Sordino, Peter W. Stacpoole, Brendan Lee, Massimo Zeviani, Nicola Brunetti-Pierri

  Science Translational Medicine06 Mar 2013 : 175ra31

  Phenylbutyrate enhances pyruvate dehydrogenase enzyme complex (PDHC) activity through inhibition of PDHC kinase, rescues the severe phenotype of a zebrafish model of PDHC deficiency, and prevents systemic lactic acidosis.

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