Disorders of the pyruvate dehydrogenase complex
- 1 June 1986
- journal article
- review article
- Published by Wiley in Journal of Inherited Metabolic Disease
- Vol. 9 (2) , 105-119
- https://doi.org/10.1007/bf01799447
Abstract
Pyruvate dehydrogenase deficiency may be a non-specific consequence of many different neurological degenerative disorders. There are also serious methodological problems in estimating the activity of this enzyme complex.Keywords
This publication has 71 references indexed in Scilit:
- Fatal Case of Pyruvate Dehydrogenase DeficiencyActa Paediatrica, 1985
- Effect of mycoplasma infection on pyruvate dehydrogenase complex activity of normal and pyruvate dehydrogenase complex-deficient fibroblastsExperimental Cell Research, 1984
- Lipoamide dehydrogenase deficiency with primary lactic acidosis: Favorable response to treatment with oral lipoic acidThe Journal of Pediatrics, 1984
- Pyruvate decarboxylating activity in extracts from pigeon liver acetone powderBiochemical Medicine, 1983
- Normal activities of hepatic pyruvate dehydrogenase and pyruvate carboxylase in Leigh's syndrome.The Tohoku Journal of Experimental Medicine, 1983
- STUDIES ON PYRUVATE CARBOXYLASE, PYRUVATE DECARBOXYLASE AND LIPOAMIDE DEHYDROGENASE IN SUBACUTE NECROTIZING ENCEPHALOMYELOPATHYActa Paediatrica, 1982
- Hyper-Endorphin Syndrome in a Child with Necrotizing EncephalomyelopathyNew England Journal of Medicine, 1980
- Defective activation of the pyruvate dehydrogenase complex in subacute necrotizing encephalomyelopathy (leigh disease)Annals of Neurology, 1979
- Methodology in evaluating pyruvate oxidationAnnals of Neurology, 1979
- Pyruvate Dehydrogenase Phosphatase DeficiencyPediatric Research, 1975