RT Journal Article
SR Electronic
T1 Pyruvate dehydrogenase phosphate (PDH<sub>b</sub>) phosphatase activity in fibroblasts from Leigh's disease
JF Neurology
JO Neurology
FD Lippincott Williams &amp; Wilkins
SP 1187
OP 1187
DO 10.1212/WNL.34.9.1187
VO 34
IS 9
A1 Sheu, Kwan-Fu Rex
A1 Blass, John P.
YR 1984
UL http://n.neurology.org/content/34/9/1187.abstract
AB 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.