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Impact of Parkin overexpression in a mouse model of Duchenne’s disease

Project description

Duchenne muscular dystrophy (DMD) is an inherited disease resulting from mutations in the gene encoding dystrophin, which leads to absence of this subsarcolemmal cytoskeletal protein from striated muscle cells. Although much progress has been made in our understanding of DMD, the precise mechanisms involved in the progression of the disease still remain unclear. The absence of dystrophin is obviously central in the pathogenesis of DMD since it renders the sarcolemma fragile and instable. Several studies in DMD patients and mdx mice (the most frequently employed animal model of the disease) reported data indicating that mitochondrial dysfunction are involved in the progression of DMD and that improving mitochondrial fitness (i.e. mitochondrial content and function) is a promising therapeutic strategy to improve muscle function in DMD. However, to date, no study has investigated whether stimulating mitophagy, the process in charge of the removal of damaged/dysfunctional mitochondria, can attenuate mitochondrial dysfunction in dystrophic muscle and slow-down the progression of DMD. The aim of the present project is therefore to define whether overexpressing Parkin, an E3 ligase regulating the recognition and removal of damaged mitochondria, can improve skeletal muscle and mitochondrial function of mdx mice.

Research Team

Name: Olivier Reynaud, M.Sc.
Supervisor:
Gilles Gouspillou (UQAM)
Laureate:
Doctoral scholarship 2019

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