Defects in the mitochondrial energy metabolism outside the respiratory chain and the pyruvate dehydrogenase complex
โ Scribed by Frans J.M. Trijbels; Wim Ruitenbeek; Marjan Huizing; Udo Wendel; Jan A.M. Smeitink; Rob C.A. Sengers
- Book ID
- 110242393
- Publisher
- Springer
- Year
- 1997
- Tongue
- English
- Weight
- 19 KB
- Volume
- 174
- Category
- Article
- ISSN
- 0300-8177
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## Abstract A new parameter is presented for considering the contribution from each complex of the mitochondrial respiratory chain to the creation of the electrochemical gradient. This parameter is the proton gradient generated in each complex per pair of electrons transported (ฮH^+^/2e^โ^) and may
Defects of the mitochondrial respiratoiy chain are increasingly being recognized as an important cause of neurological disease in humans. In many of these patients, the biochemical defect results from an abnormality of the mitochondrial genome. Respiratory chain defects involving complex 11, which i