PT  - JOURNAL ARTICLE
AU  - Sheu, Kwan-Fu Rex
AU  - Blass, John P.
TI  - Pyruvate dehydrogenase phosphate (PDH<sub>b</sub>) phosphatase activity in fibroblasts from Leigh's disease
AID  - 10.1212/WNL.34.9.1187
DP  - 1984 Sep 01
TA  - Neurology
PG  - 1187--1187
VI  - 34
IP  - 9
4099  - http://n.neurology.org/content/34/9/1187.short
4100  - http://n.neurology.org/content/34/9/1187.full
SO  - Neurology1984 Sep 01; 34
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.