Andrew studies how mitochondria, the organelles responsible for energy production, assemble and repair themselves. Mitochondria are essential for cellular life; damaged mitochondria can trigger adverse immune responses that lead to inflammation and neurodegeneration. Specifically, Andrew’s project focuses on the characterization of one enzyme within the mitochondria - the mitochondrial processing peptidase (MPP) - which is a protease that cleaves off targeting signals from proteins which are directed to and imported into mitochondria. While mutations in MPP are implicated in various disorders including cerebellar ataxias and severe childhood neurodegeneration, exactly how MPP dysfunction causes these disorders remains unclear. Moreover, there are currently no therapeutics for treating these disorders.  During the scholarship period, Andrew will focus on the biochemical characterization and structural determination of MPP in complex with substrates in order to unveil the mechanisms of MPP function and its dysregulation in disease. This work will propel the development of MPP-focused therapeutics and will shed light on novel mechanisms of mitochondrial assembly and repair in health and disease.

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Andrew détiendra la bourse et effectuera ses recherches dans le laboratoire du Dr. Jean-François Trempe (Trempe Lab | Structural pharmacology | Québec)