Impaired mitochondrial beta-oxidation in a patient with an abnormality of the respiratory chain. Studies in skeletal muscle mitochondria.

Abstract

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 severe abnormalities of complexes III and IV (less than 5, 63, and 30% of control values, respectively) in a skeletal muscle mitochondrial fraction from a 22-yr-old… (More)

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@article{Watmough1990ImpairedMB, title={Impaired mitochondrial beta-oxidation in a patient with an abnormality of the respiratory chain. Studies in skeletal muscle mitochondria.}, author={Nicholas J. Watmough and Laurence A Bindoff and Mark A Birch-Machin and Sandra Jackson and Kim Bartlett and Christopher Ragan and Joanna Poulton and R. Mark Gardiner and H. Stanley A. Sherratt and Douglass Matthew Turnbull}, journal={The Journal of clinical investigation}, year={1990}, volume={85 1}, pages={177-84} }