Monika Madej

Senior Research Associate

I have joined MRC Center for Neuromuscular diseases as a senior postdoctoral fellow with the aim to establish disease modeling laboratory. Our primary focus is on disorders caused by mitochondrial DNA mutations. Despite increasing knowledge of molecular details underpinning mitochondrial dysfunction, factors determining onset, progression and tissue heterogeneity of this systemic disorders are still not very well understood. Most studies focus on the analysis of bioenergetic impairment itself, while we would like to understand how this bioenergetic impairment is sensed by the cell and impacts its fitness.

To this end, I have developed and characterized human iPSC-derived skeletal muscle model of genetically confirmed mitochondrial diseases. Skeletal myotubes can form glycolytic or oxidative fibers and are commonly affected by mitochondrial mutations thereby can serve as a suitable in vitro model system.

It is well established that abundance and subcellular availability of the metabolic intermediates reflect the energetic state of the cell. We are particularly interested in enzymes, which utilize those intermediary metabolic products as substrates and cofactors and how modulation of their activity impacts cells’ function and re-wires the energy metabolism to meet cellular energy demand. With this aim, we hope to better understand and treat disorders caused by mitochondrial dysfunction.

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