Phenylbutyrate increases pyruvate dehydrogenase complex activity in cells harboring a variety of defects

OBJECTIVE: Deficiency of pyruvate dehydrogenase complex (PDHC) is the most common genetic disorder leading to lactic acidosis. PDHC deficiency is genetically heterogenous and most patients have defects in the X-linked E1-α gene but defects in the other components of the complex encoded by PDHB, PDHX...

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Main Authors: Ferriero, Rosa, Boutron, Audrey, Brivet, Michele, Kerr, Douglas, Morava, Eva, Rodenburg, Richard J, Bonafé, Luisa, Baumgartner, Matthias R, Anikster, Yair, Braverman, Nancy E, Brunetti-Pierri, Nicola
Format: Artigo
Language:Inglês
Published: BlackWell Publishing Ltd 2014
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Research Articles
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC4184775/
https://ncbi.nlm.nih.gov/pubmed/25356417
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/acn3.73
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