EC Number   |
Natural Substrates |
|---|
   3.4.24.B18 | carbonylated membrane proteins + H2O |
- |
| 3.4.24.B18 | Ccp1 precursor + H2O |
i.e. cytochrome c peroxidase, natural substrate |
| 3.4.24.B18 | cytochrome c peroxidase + H2O |
the m-AAA protease is enriched in the inner boundary membrane of mitochondria. The membrane-anchored precursor form of cytochrome c peroxidase (pCcp1) is preferentially localized in this subdomain of the inner membrane. On processing by the m-AAA protease and rhomboid protease Pcp1, the mature Ccp1 is released and moves into the cristae space |
| 3.4.24.B18 | cytochrome c peroxidase 1 + H2O |
cleaved at Ala29 in the middle of the hydrophobic segment by the inner membrane m-AAA protease complex |
| 3.4.24.B18 | HMG1-phosphorylated mitochondrial transcription factor A + H2O |
- |
| 3.4.24.B18 | more |
strong genetic interaction between Afg3l2 and Spg7 in adult neurons, indicating that both m-AAA protease isoenzymes are required to prevent degenerative changes. The m-AAA protease protects against cerebellar degeneration |
| 3.4.24.B18 | more |
strong genetic interaction between Afg3l2 and Spg7 in adult neurons, indicating that both m-AAA protease isoenzymes are required to prevent degenerative changes. The m-AAA protease protects against cerebellar degeneration. Loss of function of paraplegin (encoded by the SPG7 gene) causes hereditary spastic paraplegia, a disease characterized by retrograde degeneration of cortical motor axons |
| 3.4.24.B18 | more |
strong genetic interaction between Afg3l2 and Spg7 in adult neurons, indicating that both m-AAA protease isoenzymes are required to prevent degenerative changes. The m-AAA protease protects against cerebellar degeneration. Spg72/2 mice show a late-onset degeneration of long spinal and peripheral axons with accumulation of abnormal mitochondria. m-AAA protease affects mt-DNA content and stability of the respiratory complexes |
| 3.4.24.B18 | more |
m-AAA protease ensures maturation of cytochrome c peroxidase 1 (Ccp1). The precursor of Ccp1 is dislocated from the inner mitochondrial membrane to allow cleavage by the rhomboid protease Pcp1. Dislocation depends on the ATPase but not the proteolytic activity of the m-AAA protease |
| 3.4.24.B18 | more |
DNA-bound mitochondrial transcription factor A is resistant to proteolysis |
