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NEUROLOGY 1984;34:1187
© 1984 American Academy of Neurology

Pyruvate dehydrogenase phosphate (PDHb) phosphatase activity in fibroblasts from Leigh's disease

Kwan-Fu Rex Sheu, PhD and John P. Blass, MD, PhD

From the Altschul Laboratory of Dementia Research, Cornell University Medical College, the Burke Rehabilitation Center, White Plains, NY.

The defective activation of pyruvate dehydrogenase complex (PDHC) in Leigh's disease (subacute necrotizing encephalomyelopathy) could be due to deficiency of pyruvate dehydrogenase phosphate (PDH,) 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.

Address correspondence and reprint requests to Dr. Sheu, Altschul Laboratory of Dementia Research, Cornell University Medical College, The Burke Rehabilitation Center, 785 Mamaroneck Avenue, White Plains, NY 10605.

This work is supported in part by NIH grant NS15125 and grants from the Muscular Dystrophy Association, the Will Rogers Institute, the Winifred Masterson Burke Relief Foundation, the Brown and Williamson Tobacco Company, and the General Foods Corporation.

Accepted for publication January 13, 1984.






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