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

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

BACKGROUND: Coenzyme Q10 (CoQ10 or ubiquinone) deficiency can be due either to mutations in genes involved in CoQ10 biosynthesis pathway, or to mutations in genes unrelated to CoQ10 biosynthesis. CoQ10 defect is the only oxidative phosphorylation disorder that can be clinically improved after oral C...

Full description

Bibliographic Details
Main Authors: Konstantina Fragaki, Annabelle Chaussenot, Jean-François Benoist, Samira Ait-El-Mkadem, Sylvie Bannwarth, Cécile Rouzier, Charlotte Cochaud, Véronique Paquis-Flucklinger
Format: Article
Language:English
Published: BMC
Series:Biological Research
Subjects:
Mitochondrial disease
CoQ10 deficiency
Respiratory chain
Spectrophotometry
LC-MSMS
Online Access:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0716-97602016000100004&lng=en&tlng=en

Internet

http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0716-97602016000100004&lng=en&tlng=en

Similar Items