The quantification of mitochondrial enzyme activities in skeletal muscle samples of patients suspected of having mitochondrial myopathies is problematic. Therefore, we have evaluated different methods for the determination of activities cytochrome c oxidase and NADH:CoQ oxidoreductase in human skele
An Evaluation of the Measurement of the Activities of Complexes I-IV in the Respiratory Chain of Human Skeletal Muscle Mitochondria
β Scribed by M.A. Birchmachin; H.L. Briggs; A.A. Saborido; L.A. Bindoff; D.M. Turnbull
- Book ID
- 112237955
- Publisher
- Elsevier Science
- Year
- 1994
- Tongue
- English
- Weight
- 709 KB
- Volume
- 51
- Category
- Article
- ISSN
- 0885-4505
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Respiratory chain enzymes were studied in isolated mitochondria of two patients with mitochondrial myopathy. Both patients had been suffering from chronic progressive external ophthalmoplegia and abnormal muscular fatigability since late childhood. One of the patients exhibited the complete triad of
## Abstract Complex III isolated from yeast mitochondria catalyzed an antimycin A and Diuronβsensitive coenzyme QH~2~βcytochrome __c__ reductase activity with a turnover number of 15.7 sec^β1^ and contained 10 nmoles of cytochrome __b__ and 4.6 nmoles of cytochrome __c__~1~ per mg of protein. Elect