Decrease of pyruvate dehydrogenase phosphatase activity in patients with congenital lactic acidemia

Michinori Ito, Hideaki Kobashi, Etsuo Naito, Takahiko Saijo, Eiji Takeda, A. H.M.Mahbubul Huq, Yasuhiro Kuroda

Research output: Contribution to journalArticlepeer-review

13 Scopus citations

Abstract

We developed an assay method for pyruvate dehydrogenase phosphatase activity using [1-14C]pyruvate and measured pyruvate dehydrogenase phosphatase activity in cultured skin fibroblasts from three patients with congenital lactic acidemia due to a defect in activation of the pyruvate dehydrogenase complex. The enzyme activity of their fibroblasts was significantly reduced to 50.7%, 64.6% and 63.1% of that of control fibroblasts. These observations suggest that the defect in activation of the pyruvate dehydrogenase complex in these patients might be due to a reduction in pyruvate dehydrogenase phosphatase activity.

Original languageEnglish
Pages (from-to)1-7
Number of pages7
JournalClinica Chimica Acta
Volume209
Issue number1-2
DOIs
StatePublished - Jul 31 1992

Keywords

  • Deficiency of pyruvate dehydrogenase complex
  • Lactic acidemia
  • Pyruvate dehydrogenase
  • Pyruvate dehydrogenase phosphatase

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