Application | Comment | Organism |
---|---|---|
medicine | in muscle creatine kinase mouse heart model for Friedreich ataxia, a rare hereditary neurodegenerative disease characterized by progressive ataxia and cardiomyopathy, there is a clear progressive increase in protein levels of mitochondrial ATP-dependent proteases, Lon and ClpP, in the hearts of muscle creatine kinase mutants. Lon and ClpP upregulation, which is triggered at a mid-stage of the disease through separate pathways, is accompanied by an increase in proteolytic activity. There is a simultaneous and significant progressive loss of mitochondrial Fe-S proteins with no substantial change in their mRNA level | Mus musculus |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | - |
Mus musculus | 5739 | - |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Mus musculus | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
heart | - |
Mus musculus | - |
Organism | Comment | Expression |
---|---|---|
Mus musculus | in muscle creatine kinase mouse heart model for Friedreich ataxia, a rare hereditary neurodegenerative disease characterized by progressive ataxia and cardiomyopathy, there is a clear progressive increase in protein levels of mitochondrial ATP-dependent proteases, Lon and ClpP, in the hearts of muscle creatine kinase mutants. Lon and ClpP upregulation, which is triggered at a mid-stage of the disease through separate pathways, is accompanied by an increase in proteolytic activity. | up |