
Picture of Orfi Zakaria
Project description
Duchenne muscular dystrophy (DMD) is an inherited disease resulting from mutations in the gene encoding dystrophin, causing the absence of this protein from striated muscle cells. Although much progress has been made in our understanding of DMD, the precise mechanisms involved its progression still remain unclear. Studies in DMD patients and mdx mice (the most studied animal model of DMD) have shown that muscles lacking dystrophin display mitochondrial dysfunction that contributes to the development of the disease. The maintenance of mitochondrial health relies on the subtle coordination of processes involved in mitochondrial biogenesis and mitochondrial quality control processes such as mitophagy -a process in charge of the removal of dysfunctional mitochondria, regulated in part by Parkin. While previous studies have shown that stimulating mitochondrial biogenesis can positively impact disease progression in mdx mice, no study has investigated whether stimulating mitophagy can also attenuate the progression of DMD. In this setting, the present project aims at defining whether stimulating mitophagy by overexpressing Parkin can improve skeletal muscle and mitochondrial function of mdx mice. The present project could therefore identify Parkin as a novel potential therapeutic target to slow down the progression of DMD.
Research team
Principal investigator: Pr Gilles Gouspillou (UQAM)
Collaborator: Pr Marc Lussier
Laureate: 2019 Collaborative Research Grant
