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Julien Beyrath

WP3: Model validation, refinement and application

Action line: Evaluation of the effect of model-predicted pharmaceutical targets for complex I deficiency at relevant level of complexity.

The main objective of this action line is to obtain new knowledge about the effect of model-predicted pharmaceutical targets to modulate complex I activity in skeletal muscle cells and mice in vivo.
Modulators of the predicted targets will be developed (rational design, phage display, antibodies production…) or selected from literature and tested in vitro for their ability to restore defective pathways in mitochondrial complex I deficient cells. Positive hits will then be tested in vivo on the NDUFS4 knock out mouse model which mimics in great detail the human disease, thus representing a clinically relevant model.

Personal profile:
Julien obtained his M.Sc in Pharmacology (2006) and his PhD in molecular and cellular biology (2010) from the University of Strasbourg, France. The major focus of his research was directed to the development and the study of synthetic ligands targeting the TNFR (Tumor-Necrosis-Factor Receptors) and TLR (Toll-Like Receptors) families with the aim of using these reagents as potential cancer therapeutic agents. He now dedicates his research toward the identification of new drug targets for mitochondrial diseases. Since January 2013 Julien also holds a position at Khondrion.