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  • Title: Normal activities of hepatic pyruvate dehydrogenase and pyruvate carboxylase in Leigh's syndrome.
    Author: Tsuchiyama A, Oyanagi K, Sogawa H, Nakao T, Ogawa K, Fujita S.
    Journal: Tohoku J Exp Med; 1983 Jan; 139(1):67-72. PubMed ID: 6836560.
    Abstract:
    A case of Leigh's syndrome (subacute necrotizing encephalomyelopathy, SNE), proven by autopsy, was reported. The persistent elevation of pyruvate and lactate in blood and hyperalanemia suggested an impairment of pyruvate oxidation, but the enzyme activities of pyruvate dehydrogenase (PDH) and pyruvate carboxylase (PC) in liver tissues of the patient revealed normal. It is postulated that Leigh's syndrome and both enzyme deficiencies are distinct entities.
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