MIPEP
Description
The MIPEP (mitochondrial intermediate peptidase) is a protein-coding gene located on chromosome 13.
Mitochondrial intermediate peptidase is an enzyme encoded by the MIPEP gene in humans. This protein plays a critical role in the mitochondrial protein import machinery, specifically in the maturation process of nuclear-encoded mitochondrial proteins with a mitochondrial translocation peptide. These proteins are often involved in oxidative phosphorylation (OXPHOS). The MIPEP gene is highly expressed in the heart, skeletal muscle, and pancreas, organs often affected by OXPHOS disorders. The protein, with a size of 80.6 kDa and 713 amino acids, contains a mitochondrial targeting peptide (amino acids 1-35). MIPEP works in collaboration with general mitochondrial processing peptidase (MPP) to mature a specific class of nuclear-encoded precursor proteins. MPP initially cleaves these precursors, leaving an octapeptide at the protein's N-terminus. Subsequently, MIPEP cleaves this octapeptide, completing the final maturation process.
MIPEP is also known as COXPD31, HMIP, MIP.
