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Literature summary for 3.4.21.53 extracted from

  • Guillon, B.; Bulteau, A.L.; Wattenhofer-Donze, M.; Schmucker, S.; Friguet, B.; Puccio, H.; Drapier, J.C.; Bouton, C.
    Frataxin deficiency causes upregulation of mitochondrial Lon and ClpP proteases and severe loss of mitochondrial Fe-S proteins (2009), FEBS J., 276, 1036-1047.
    View publication on PubMed

Application

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

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
Mus musculus 5739
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Organism

Organism UniProt Comment Textmining
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
heart
-
Mus musculus
-

Expression

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