Pyruvate dehydrogenase phosphate (PDH b ) phosphatase activity in fibroblasts from Leigh's disease
- 1 September 1984
- journal article
- Published by Wolters Kluwer Health in Neurology
- Vol. 34 (9) , 1187
- https://doi.org/10.1212/wnl.34.9.1187
Abstract
The defective activation of pyruvate dehydrogenase complex (PDHC) in Leigh9s 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, Leigh9s lines, spinocerebellar ataxias, or other neurologic disorders. The results do not support the idea that PDHb phosphatase is deficient in Leigh9s disease.Keywords
This publication has 0 references indexed in Scilit: