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Mitochondria: structure and function.
Mitochondria are the main site of ATP synthesis in aerobic cells, using the free energy of the oxidation of metabolic fuels by oxygen. They have a matrix space containing the enzymes of the citrateExpand
Fatal Lactic Acidosis in Infancy with a Defect of Complex III of the Respiratory Chain
ABSTRACT: We report our studies on the metabolic defects which caused a newborn infant to present with a severe lactic acidemia (25 mM) and to die on the 3rd d after birth despite intensiveExpand
A systematic pharmacological examination has been made of 55 Jamaican plants, most of which have a local reputation as medicinals. A number of interesting, but no outstanding, activities wereExpand
The effects of valproate on intermediary metabolism in isolated rat hepatocytes and intact rats.
Valproate is a valuable anticonvulsant which is associated with hepatotoxicity in some patients. In concentrations in the range found in man during valproate therapy (0.1-1.0 mM), it inhibitedExpand
The synthesis of hippurate from benzoate and glycine by rat liver mitochondria. Submitochondrial localization and kinetics.
1. Rat liver mitochondria make hippurate at up to 4 nmol/min per mg of protein. The rate of synthesis supported by oxidation of glutamate with exogenous Pi present is identical with that supported byExpand
Short-chain acyl-CoA dehydrogenase deficiency associated with a lipid-storage myopathy and secondary carnitine deficiency.
ENGEL and Angelini have reported that skeletal-muscle carnitine deficiency is associated with a lipid-storage myopathy.1 Since then, two major clinical syndromes have been described2: a systemic fo...
Impaired mitochondrial beta-oxidation in a patient with an abnormality of the respiratory chain. Studies in skeletal muscle mitochondria.
Defects of complex I of the mitochondrial respiratory chain are important causes of neurological disease. We report studies that demonstrate a severe deficiency of complex I activity with less severeExpand
Substituted 2-oxiranecarboxylic acids: a new group of candidate hypoglycaemic drugs.
Drugs to treat diabetes that can be taken orally have long been sought, although the successful management of insulin-dependent diabetes mellitus by simple chemotherapy may be an unachievable goal.Expand
Stereospecificity of the inhibition by etomoxir of fatty acid and cholesterol synthesis in isolated rat hepatocytes.
The racemates of substituted 2-oxiranecarboxylates are potent inhibitors of fatty acid oxidation and fatty acid and cholesterol synthesis. We show in the accompanying paper [Agius L, Peak M andExpand