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INSTITUTE OF PHYSIOLOGY CAS

Cutting-edge science for health

Functional characterization of ATP synthase deficiencies


ATP produced by the mitochondrial FoF1-ATP synthase represents a major source of energy for aerobic organisms. The proposed project is aimed to shedding light on the functional consequences of ATP synthase deficiencies using a model of knock-down of small subunits of the catalytic F1 part of the mammalian ATP synthase (γ, δ and ε).

To shed light on the functional consequences of ATP synthase deficiencies, we utilised a model of HEK293 cell line and explored the effect of RNAi mediated knockdown of the three subunits (γ, δ and ε) forming the central stalk of the enzyme connecting Fo and F1 domains. Using these model clones, we are planning on investigating the effect of ATP synthase deficiency on the mitochondrial energetics, oxidative stress, energy state, and cell viability and define the threshold residual activity of ATP synthase for the presentation of pathological phenotype.

 

This project is supported by the Grant Agency of Charles University (grant 1160214) and the Czech Science Foundation (grant P303/12/1363).