Mitochondrial proteins – import, export, degradation

Abstract

Mitochondria participate in plethora of vital processes in the cell such as energy production, other biochemical pathways and signaling. Over a thousand proteins co-operate to form the proteome of mitochondria. A great majority of mitochondrial precursor proteins are encoded in nuclear DNA and produced in the cytosol. They are targeted to mitochondria and sorted to distinct sub-compartments of mitochondria with the help of specialized translocase machineries. Biogenesis of mitochondrial proteins is completed through precursor maturation events and complex assembly. Mitochondrial homeostasis also requires the presence of clearance mechanisms for degradation of non-functional proteins. In the present review, we summarize the most important aspects of mitochondrial protein biogenesis.