Workshops & seminars

Deciphering the biochemical consequences of mitochondrial dysfunction in diseases using metabolomics
Dr. Christine Des Rosiers(Universite de Montreal)

Friday, April 13, 2018
2:30 p.m. – 4 p.m.

Dr. Christine Des Rosiers


This event is free



Hingston Hall, wing HC
7141 Sherbrooke W.
Room HC-155



Abstract:  Impaired mitochondrial respiration underlies inborn errors of metabolism and age-associated diseases. Inherited mitochondrial disorders (prevalence: 1:5000) are associated with a wide spectrum of clinical manifestations and a high mortality rate, mostly in childhood. While impaired oxidative phosphorylation is a major pathogenic mechanism, a better understanding of the resulting perturbations in energy nutrient metabolism beyond ATP is essential to the optimal management of patients and design of potential interventions, which are currently inexistent. The recent emergence of metabolomics technologies offers a means to systematically measure thousands of low-molecular weight compounds in order to provide a global view of alterations in metabolic pathways induced by a given perturbations, whether resulting from a gene mutation or disease onset. This presentation will first provide an overview of the promise and challenges of metabolomics as applied to biomedical research. Then, it will also discuss results from our recent work in which metabolomics was applied to humans and mice carrying LRPPRC-dependent mitochondrial dysfunction, which is the root cause of Leigh syndrome French Canadian variant (LSFC).

Bio:  Dr. Christine Des Rosiers is a Professor in the Department of Nutrition of the Université de Montréal and Director of the Montreal Heart Institute Research Centre Metabolomic Laboratory and Platform.  She is a founding member of the Society for Heart and Vascular Metabolism and is currently serving as President since 2015. The focus of her research is on the role of metabolic alterations in the pathogenesis of disease, particularly heart disease. She has over 25 years of research experience in metabolic investigations using stable isotopes and mass spectrometric-based methodology. She specifically gained recognition for the development of these methods for the metabolic and functional phenotyping of the ex vivo working mouse heart. More recently, she has taken the direction of metabolomic initiatives as part of multidisciplinary translational projects aiming at the discovery of biomarkers of disease development or treatment response in various conditions, which include heart disease, but also diabetes, as well as mitochondrial and inflammatory diseases.


Dr. Johnson is the guest of Dr. Dajana Vuckovic

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