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Mammalian mitochondrial beta-oxidation.
The enzymic stages of mammalian mitochondrial beta-oxidation were elucidated some 30-40 years ago. However, the discovery of a membrane-associated multifunctional enzyme of beta-oxidation, aExpand
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Mitochondrial beta-oxidation.
Mitochondrial beta-oxidation is a complex pathway involving, in the case of saturated straight chain fatty acids of even carbon number, at least 16 proteins which are organized into two functionalExpand
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The specificity of glycine-N-acylase and acylglycine excretion in the organicacidaemias.
The specificity of the enzyme glycine-N-acylase prepared from ox liver mitochondria for the coenzyme A derivatives of several organic acids has been investigated. The kinetic constants of this enzymeExpand
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Mammalian mitochondrial β-oxidation
The enzymic stages of mammalian mitochondrial β-oxidation were elucidated some 30–40 years ago. However, the discovery of a membrane-associated multifunctional enzyme of β-oxidation, aExpand
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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
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Combined enzyme defect of mitochondrial fatty acid oxidation.
A young girl presented with recurrent episodes of muscle weakness culminating in a severe attack of generalized muscle weakness. In the muscle mitochondria from the patient there was an abnormalExpand
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The Factorial Survey: An Approach to Defining Sexual Harassment on Campus
A factorial survey was used to assess perceptions of sexual harassment in the context of faculty-student relations in a university setting. Results of a survey of undergraduate students and facultyExpand
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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...
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A combined defect of three mitochondrial carboxylases presenting as biotin-responsive 3-methylcrotonyl glycinuria and 3-hydroxyisovaleric aciduria.
A child with a history of episodes of metabolic acidosis was found to excrete 3-hydroxyisovaleric acid and 3-methylcrotonylglycine. These metabolites disappeared following the administration ofExpand
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The synthesis and characterisation of 2-methylacetoacetyl coenzyme A and its use in the identification of the site of the defect in 2-methylacetoacetic and 2-methyl-3-hydroxybutyric aciduria.
(1) 2-Methylacetoacetyl coenzyme A was prepared, purified by ion exchange chromatography as judged by TLC and HPLC and a number of its properties characterised. (2) An assay of 3-oxothiolase inExpand
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