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Target intelligence / Profile preview
Mitochondrial intermediate peptidase (MIPEP)
- Target
- MIPEP
- Molecular classification
- Enzyme, Metalloprotease, Mitochondrial processing peptidase
01
Overview
Mitochondrial intermediate peptidase (MIPEP) is a nuclear-encoded metalloprotease located in the mitochondrial matrix, essential for the secondary proteolytic processing of a specific subset of imported mitochondrial precursor proteins[1][3]. These proteins, initially cleaved by the mitochondrial processing peptidase (MPP), require further maturation by MIPEP, which removes an additional octapeptide from their N-termini, a step necessary for their proper folding and ultimate functionality within the mitochondrion[1][2][3]. MIPEP is highly expressed in tissues with high metabolic demand (heart, skeletal muscle, pancreas), and its disruption is linked to severe mitochondrial diseases, including forms of cardiomyopathy and syndromic metabolic diseases[1]. MIPEP does not currently have any known small-molecule interacting drugs or established biomarker applications in clinical settings[1][3].
- Other names
- MIPEPHMIPMIPCOXPD31mitochondrial intermediate peptidase
02
Biological functions
Mitochondrial protein maturationProtein import into mitochondriaProteolytic processing of precursor proteinsRegulation of oxidative phosphorylation (OXPHOS)-related proteins
03
Disease associations
Mitochondrial diseaseCardiomyopathy (particularly left ventricular non-compaction)Association with metabolic dysfunction and obesityPotential link to high myopia (but not conclusively established)
04
Safety considerations
Pathogenic *MIPEP* variants can lead to early childhood or infantile disease including hypotonia, progressive cardiomyopathy, and early deathDysfunction can also contribute to metabolic alterations in adipose tissue
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