Sheu K F, Blass J P
Neurology. 1984 Sep;34(9):1187-91. doi: 10.1212/wnl.34.9.1187.
The defective activation of pyruvate dehydrogenase complex (PDHC) in Leigh's disease (subacute necrotizing encephalomyelopathy) could be due to deficiency of pyruvate dehydrogenase phosphate (PDHb) phosphatase (EC 3.1.3.43). This enzyme catalyzes the dephosphorylation and activation of phospho-PDHC. In cultured skin fibroblasts, we assayed this enzyme by measuring the rate of activation of the exogenously added, purified phospho-PDHC (bovine kidney). PDHb phosphatase activity did not differ significantly among normal control cells, Leigh's lines, spinocerebellar ataxias, or other neurologic disorders. The results do not support the idea that PDHb phosphatase is deficient in Leigh's disease.
Leigh病（亚急性坏死性脑脊髓病）中丙酮酸脱氢酶复合体（PDHC）的缺陷性激活可能是由于丙酮酸脱氢酶磷酸酯（PDHb）磷酸酶（EC 3.1.3.43）缺乏所致。该酶催化磷酸化PDHC的去磷酸化及激活。在培养的皮肤成纤维细胞中，我们通过测量外源添加的纯化磷酸化PDHC（牛肾）的激活速率来检测这种酶。PDHb磷酸酶活性在正常对照细胞、Leigh病细胞系、脊髓小脑共济失调症或其他神经系统疾病之间无显著差异。这些结果不支持Leigh病中存在PDHb磷酸酶缺乏的观点。
