Focal deficiency of cytochrome c oxidase and of mitochondrial ATPase with histochemical evidence of loosely coupled oxidative phosphorylation in a mitochondrial myopathy of a patient with bilateral ptosis

1985 ◽  
Vol 69 (1-2) ◽  
pp. 27-36 ◽  
Author(s):  
J. Müller-Höcker ◽  
S. Stünkel ◽  
D. Pongratz ◽  
G. Hübner
Keyword(s):  
Cytochrome C ◽  
Oxidative Phosphorylation ◽  
Cytochrome C Oxidase ◽  
Mitochondrial Myopathy ◽  
Mitochondrial Atpase ◽  
Bilateral Ptosis ◽  
Loosely Coupled ◽  
Histochemical Evidence

Mitochondrial myopathy due to novel missense mutation in the cytochrome c oxidase 1 gene

2012 ◽  
Vol 319 (1-2) ◽  
pp. 158-163 ◽  
Author(s):  
Rami Massie ◽  
Jing Wang ◽  
Li-Chieh Chen ◽  
Victor W. Zhang ◽  
Michael P. Collins ◽  
...  
Keyword(s):  
Cytochrome C ◽  
Missense Mutation ◽  
Cytochrome C Oxidase ◽  
Mitochondrial Myopathy

scholarly journals Proteomics of Cytochrome c Oxidase-Negative versus -Positive Muscle Fiber Sections in Mitochondrial Myopathy

Cell Reports ◽  
2019 ◽  
Vol 29 (12) ◽  
pp. 3825-3834.e4 ◽  
Author(s):  
Marta Murgia ◽  
Jing Tan ◽  
Philipp E. Geyer ◽  
Sophia Doll ◽  
Matthias Mann ◽  
...  
Keyword(s):  
Cytochrome C ◽  
Cytochrome C Oxidase ◽  
Muscle Fiber ◽  
Mitochondrial Myopathy

Fatal infhntile mitochondrial myopathy and renal dysfunction due to cytochrome-c-oxidase deficiency

Neurology ◽  
1980 ◽  
Vol 30 (8) ◽  
pp. 795-795 ◽  
Author(s):  
S. DiMauro ◽  
J. R. Mendell ◽  
Z. Sahenk ◽  
D. Bachman ◽  
A. Scarpa ◽  
...  
Keyword(s):  
Cytochrome C ◽  
Renal Dysfunction ◽  
Cytochrome C Oxidase ◽  
Mitochondrial Myopathy ◽  
Oxidase Deficiency ◽  
Cytochrome C Oxidase Deficiency

scholarly journals Oxidative phosphorylation during glycollate metabolism in mitochondria from phototrophic Euglena gracilis

1975 ◽  
Vol 150 (3) ◽  
pp. 373-377 ◽  
Author(s):  
N Collins ◽  
R H Brown ◽  
M J Merrett
Keyword(s):  
Cytochrome C ◽  
Oxygen Uptake ◽  
Oxidative Phosphorylation ◽  
Cytochrome C Oxidase ◽  
Electron Acceptor ◽  
Euglena Gracilis ◽  
Gradient Centrifugation ◽  
Antimycin A ◽  
Isolated Mitochondria ◽  
Glycollate Dehydrogenase

Mitochondria were isolated by gradient centrifugation on linear sucrose gradients from broken cell suspensions of phototrophically grown Euglena gracilis. An antimycin A-sensitive but rotenone-insensitive glycollate-dependent oxygen uptake was demonstrated in isolated mitochondria. The partial reactions of glycollate-cytochrome c oxidoreductase and cytochrome c oxidase were demonstrated by using Euglena cytochrome c as exogenous electron acceptor/donor. Isolated mitochondria contain glycollate dehydrogenase and glyoxylate-glutamate aminotransferase and oxidize exogenous glycine. A P:O ratio of 1.7 was obtained for glycollate oxidation, consistent with glycollate electrons entering the Euglena respiratory chain at the flavoprotein level. The significance of these results is discussed in relation to photorespiration in algae.

scholarly journals The mechanism for the ATP-induced uncoupling of respiration in mitochondria of the yeast Saccharomyces cerevisiae

1995 ◽  
Vol 307 (3) ◽  
pp. 657-661 ◽  
Author(s):  
S Prieto ◽  
F Bouillaud ◽  
E Rial
Keyword(s):  
Saccharomyces Cerevisiae ◽  
Membrane Potential ◽  
Cytochrome C ◽  
Oxidative Phosphorylation ◽  
Cytochrome C Oxidase ◽  
Kinetic Properties ◽  
Yeast Saccharomyces Cerevisiae ◽  
Saccharomyces Cerevisiae Mitochondria ◽  
Low Efficiency ◽  
Respiratory Chain Activity

We have recently reported that ATP induces an uncoupling pathway in Saccharomyces cerevisiae mitochondria [Prieto, Bouillaud, Ricquier and Rial (1992) Eur. J. Biochem. 208, 487-491]. The presence of this pathway would explain the reported low efficiency of oxidative phosphorylation in S. cerevisiae, and may represent one of the postulated energy-dissipating mechanisms present in these yeasts. In this paper we demonstrate that ATP exerts its action in two steps: first, at low ATP/Pi ratios, it increases the respiratory-chain activity, probably by altering the kinetic properties of cytochrome c oxidase. Second, at higher ATP/Pi ratios, an increase in membrane permeability leads to a collapse in membrane potential. The ATP effect on cytochrome c oxidase corroborates a recent report showing that ATP interacts specifically with yeast cytochrome oxidase, stimulating its activity [Taanman and Capaldi (1993) J. Biol. Chem. 268, 18754-18761].

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