Dr Bémeur is co-author of an article publish today in PLosONE journal.
This article demonstrated mitochondrial deficiencies observed in fibroblasts from young patients with Leigh syndrome (Canadian-French form). They were able to show that these fibroblasts are unable to sustain a nutritional stress; fatty acids acting as a precipitant in fatal lactic acidosis crisis in patients. Various therapeutic strategies targeting mitochondria or modulating fatty acids were beneficial. However, antioxidants showed no positive effect, even enhancing fibroblasts cell death.
Dr Bémeur is currently pursuing this project, by studying inflammatory stress with a grant of Grand Défi Pierre-Lavoie.
Article is freely available on editor's website.
Mitochondrial vulnerability and increased susceptibility to nutrient-induced cytotoxicity in fibroblasts from leigh syndrome French canadian patients.
Yan Burelle, Chantal Bémeur, Marie-Eve Rivard, Julie Thompson Legault, Gabrielle Boucher, L. S. F. C. Consortium, Charles Morin, Lise Coderre, Christine Des Rosiers.