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    Chapter

    Two Cases of ß-KetothioIase Deficiency: A Comparison

    At least three isoenzymes of ß-ketothiolase exist in human fibroblasts: (1) a mitochondrial’ branched chain’ ß-ketothiolase (MBK) (EC 2.3.1.9) which can thiolyse 2-methylacetoacetyl CoA and acetoacetyl CoA., (...

    B. Middleton, R. G. F. Gray, M. J. Bennett in Organic Acidurias (1984)

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    Chapter

    The Diagnosis and Biochemical Investigation of a Patient with a Short Chain Fatty Acid Oxidation Defect

    Though the clinical and biochemical features of medium chain acyl-CoA dehydrogenase deficiency are now becoming well established we have much less experience of defects in short chain acyl-CoA dehydrogenation....

    M. J. Bennett, R. G. F. Gray… in Inherited Disorders of Vitamins and Cofact… (1985)

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    Chapter

    Generalised Dicarboxylic Aciduria: A Common Finding in Neonates

    Inborn errors of organic acid metabolism presenting with sudden onset hypoglycaemia in association with infection or prolonged fasting have been shown to account for a proportion of sudden infant deaths (SID) ...

    M. Downing, P. Rose, M. J. Bennett, N. J. Manning… in Studies in Inherited Metabolic Disease (1989)