Mitochondrial proteases in human diseases

Mitochondria contain more than 1000 different proteins, including several proteolytic enzymes. These mitochondrial proteases form a complex system that performs limited and terminal proteolysis to build the proteome, maintain, control its functions or degrade proteins peptides. During protein biogenesis, presequence cleave targeting signals obtain mature functional proteins. Processing by also exerts regulatory role in modulation of quality enzymes misfolded, aged, superfluous Depending on their substrates, defects can affect majority proteome only single protein. Consequently, mutations have been linked human diseases. This review gives an overview components machinery highlights pathological consequences dysfunctional processing turnover.