Mitochondria are semi-autonomous, sub-cellular organelles essential for living cells. They are separated from the surrounding cytosol by a specialized double membrane and contain their own genome present in multiple copies and organized into structures termed 'nucleoids' which are associated with the inner mitochondrial membrane.
The mitochondrial genome encodes 13 highly hydrophobic proteins which contribute to the membrane-bound electron transport chain, as well as 2 rRNAs and 22 tRNAs essential for their translation by the mitoribosomes. All other proteins of the mitochondrial proteome, which is currently estimated to contain more than 1000 proteins, are encoded in the nucleus and are imported into mitochondria through specialized membrane transporters.
Mitochondria perform multiple functions in mammalian cells, including the production of ATP through oxidative phosphorylation, regulation of intracellular calcium homeostasis, control of apoptosis, and biosynthesis of key macromolecules essential for cell proliferation and differentiation. Mitochondrial dysfunction is associated with a wide range of human diseases.
Research in our lab is currently focused in three main areas