Mitochondrial Processing Peptidases-Structure, Function and the Role in Human Diseases.

ABSTRACT: Mitochondrial proteins are encoded by both nuclear and mitochondrial DNA. While some of the essential subunits of the oxidative phosphorylation (OXPHOS) complexes responsible for cellular ATP production are synthesized directly in the mitochondria, most mitochondrial proteins are first translated in the cytosol and then imported into the organelle using a sophisticated transport system. These proteins are directed mainly by targeting presequences at their N-termini. These presequences need to be cleaved to allow the proper folding and assembly of the pre-proteins into functional protein complexes. In the mitochondria, the presequences are removed by several processing peptidases, including the mitochondrial processing peptidase (MPP), the inner membrane processing peptidase (IMP), the inte

SUBMITTER: Kunova N 

PROVIDER: S-EPMC8835746 | biostudies-literature | 2022 Jan

REPOSITORIES: biostudies-literature