EC Number |
General Information |
Reference |
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3.4.24.64 | malfunction |
ablation of the enzyme not only affects processing of mitochondrial matrix proteins but also inhibits the import process itself |
734654 |
3.4.24.64 | malfunction |
analysis of the mas1 mutant (temperature-sensitive mas1 mutant strain in which mitochondrial processing protease (MPP) is inactive when the cells are shifted to growth at nonpermissive temperature (37 °C)) reveales an increase in protein levels of nonprocessed mitochondrial proteins as well as an elevated membrane potential and increased oxygen consumption in mas1 mitochondria upon growth at nonpermissive temperature is observed. These findings might point toward a response in mas1 cells triggered to counterbalance the proteotoxic stress induced by MPP dysfunction |
-, 754485 |
3.4.24.64 | malfunction |
mutations in the substrate binding glycine-rich loop of the mitochondrial processing peptidase-alpha protein (PMPCA) cause a severe mitochondrial disease |
753318 |
3.4.24.64 | malfunction |
reducing enzyme activity induces phosphatase and tensin homologue-induced kinase 1 accumulation at the mitochondrial surface, leading to Parkin recruitment and mitophagy |
733730 |
3.4.24.64 | metabolism |
the enzyme plays a role in phosphatase and tensin homologue-induced kinase 1 (PINK1) import and mitochondrial quality control via the PINK1-Parkin pathway |
733730 |
3.4.24.64 | metabolism |
the thylakoid-transfer signal is required for energy-dependent translocation of preproteins into the thylakoid lumen and is removed by the thylakoidal processing peptidase |
733864 |
3.4.24.64 | metabolism |
yeast Nfs1 undergoes two steps of proteolytic processing: first it is cleaved by the mitochondrial processing peptidase, MPP, which removes its mitochondrial targeting sequence, and then it is cleaved by a peptidase, designated Icp55, which removes three amino acids from its N-terminus |
709066 |
3.4.24.64 | more |
expression of mppA is developmentally regulated |
709853 |
3.4.24.64 | physiological function |
most important processing enzyme that acts on proteins directed to the inner membrane intermembrane space or mitochondrial matrix. Most of the proteins targeted to the mitochondrial matrix or inner membrane present a single cleavage by mitochondrial processing peptidase |
753580 |
3.4.24.64 | physiological function |
MPP is involved in the transport of nuclear-encoded proteins from the cytosol into mitochondria |
-, 710508 |