Clinical Presentation, Genetic Etiology, and Coenzyme Q10 Levels in 55 Children with Combined Enzyme Deficiencies of the Mitochondrial Respiratory Chain

2021 ◽  
Vol 228 ◽  
pp. 240-251.e2
Author(s):  
Karin Naess ◽  
Helene Bruhn ◽  
Henrik Stranneheim ◽  
Christoph Freyer ◽  
Rolf Wibom ◽  
...  
Keyword(s):  
Respiratory Chain ◽  
Coenzyme Q10 ◽  
Clinical Presentation ◽  
Mitochondrial Respiratory Chain ◽  
Genetic Etiology ◽  
Mitochondrial Respiratory

Coenzyme Q: potentially useful index of bioenergetic and oxidative status of spermatozoa

1995 ◽  
Vol 41 (2) ◽  
pp. 217-219 ◽  
Author(s):  
A G Angelitti ◽  
L Colacicco ◽  
C Callà ◽  
M Arizzi ◽  
S Lippa
Keyword(s):  
Respiratory Chain ◽  
Coenzyme Q10 ◽  
Coenzyme Q ◽  
Mitochondrial Respiratory Chain ◽  
Oxidative Status ◽  
Atp Production ◽  
The Difference ◽  
Autoregulatory Mechanism ◽  
Infertile Patients ◽  
Mitochondrial Respiratory

Abstract The concentration of coenzyme Q10 (CoQ10), a key intermediate of the mitochondrial respiratory chain, was determined in spermatozoa of 13 fertile subjects, 8 potentially fertile patients, and 12 infertile patients. CoQ10 concentrations were significantly higher (P < 0.001) in infertile patients than in fertile and potentially fertile subjects. The difference between potentially fertile and fertile subjects was also significant (P < 0.001). We propose that a decrease in consumption of CoQ10 in both infertile and potentially fertile populations is due to an autoregulatory mechanism of ATP production.

scholarly journals Vitamin K2 cannot substitute Coenzyme Q10 as electron carrier in the mitochondrial respiratory chain of mammalian cells

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Cristina Cerqua ◽  
Alberto Casarin ◽  
Fabien Pierrel ◽  
Luis Vazquez Fonseca ◽  
Giampiero Viola ◽  
...  
Keyword(s):  
Respiratory Chain ◽  
Coenzyme Q10 ◽  
Mammalian Cells ◽  
Mitochondrial Respiratory Chain ◽  
Vitamin K2 ◽  
Electron Carrier ◽  
Mitochondrial Respiratory

scholarly journals Coenzyme Q10 defects may be associated with a deficiency of Q10-independent mitochondrial respiratory chain complexes

2016 ◽  
Vol 49 (1) ◽  
Author(s):  
Konstantina Fragaki ◽  
Annabelle Chaussenot ◽  
Jean-François Benoist ◽  
Samira Ait-El-Mkadem ◽  
Sylvie Bannwarth ◽  
...  
Keyword(s):  
Respiratory Chain ◽  
Coenzyme Q10 ◽  
Mitochondrial Respiratory Chain ◽  
Respiratory Chain Complexes ◽  
Chain Complexes ◽  
Mitochondrial Respiratory

3. Systematic Evaluation of Muscle Coenzyme Q10 Content in Children with Mitochondrial Respiratory Chain Enzyme Deficiencies

Mitochondrion ◽  
2009 ◽  
Vol 9 (1) ◽  
pp. 61-62
Author(s):  
Michael V. Miles ◽  
Lili Miles ◽  
Peter H. Tang ◽  
Paul S. Horn ◽  
Paul E. Steele ◽  
...  
Keyword(s):  
Respiratory Chain ◽  
Coenzyme Q10 ◽  
Mitochondrial Respiratory Chain ◽  
Systematic Evaluation ◽  
Respiratory Chain Enzyme ◽  
Mitochondrial Respiratory
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