The pleiotropic effects of decanoic acid treatment on mitochondrial function in fibroblasts from patients with complex I deficient Leigh syndrome

• Published in: Journal of inherited metabolic disease Vol. 39; no. 3; pp. 415 - 426
• Main Authors: Kanabus, Marta, Fassone, Elisa, Hughes, Sean David, Bilooei, Sara Farahi, Rutherford, Tricia, Donnell, Maura O’, Heales, Simon J. R., Rahman, Shamima
• Format: Journal Article
• Language: English
• Published: Dordrecht Springer Netherlands 01.05.2016; Blackwell Publishing Ltd
• Subjects: Biochemistry; Cells, Cultured; Decanoic Acids - pharmacology; Diet, Ketogenic - methods; Electron Transport Complex I - metabolism; Fatty Acids - metabolism; Fibroblasts - drug effects; Fibroblasts - metabolism; Gene Expression - drug effects; Glucose - metabolism; Human Genetics; Humans; Internal Medicine; Leigh Disease - drug therapy; Leigh Disease - metabolism; Medicine; Medicine & Public Health; Metabolic Diseases; Mitochondria - drug effects; Mitochondria - metabolism; Mitochondrial Diseases - drug therapy; Mitochondrial Diseases - metabolism; Original; Original Article; Pediatrics

There is growing interest in the use of the ketogenic diet (KD) to treat inherited metabolic diseases including mitochondrial disorders. However, neither the mechanism whereby the diet may be working, nor if it could benefit all patients with mitochondrial disease, is known. This study focusses on d...