Activating Compound | Comment | Organism | Structure |
---|---|---|---|
ATP | stimulates proteolytic activity of ClpP in heart mitochondria of muscle creatine kinase mutants | Mus musculus | |
additional information | frataxin deficiency causes significant upregulation of both mitochondrial Lon and ClpP proteases in the cardiac mouse model for Friedreich ataxia. ClpP protein level is progressively enhanced, with ca. 3fold, 3.5fold and 4.5fold increases at 5, 7 and 10 weeks of age in muscle creatine kinase mutants, respectively, despite no change in mRNA levels | Mus musculus |
Application | Comment | Organism |
---|---|---|
medicine | correlative effect of Lon and ClpP upregulation on loss of mitochondrial Fe-S proteins during the progression of Friedreich ataxia suggesting that Fe-S proteins are potential targets of Lon and ClpP proteases in Friedreich ataxia | Mus musculus |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | - |
Mus musculus | 5739 | - |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Mus musculus | O88696 | muscle creatine kinase mice | - |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
heart | - |
Mus musculus | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
casein-fluorescein isothiocyanate + H2O | - |
Mus musculus | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
ClpP Protease | - |
Mus musculus |