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Literature summary for 3.4.21.92 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

Activating Compound

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

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

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

Organism UniProt Comment Textmining
Mus musculus O88696 muscle creatine kinase mice
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Source Tissue

Source Tissue Comment Organism Textmining
heart
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Mus musculus
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
casein-fluorescein isothiocyanate + H2O
-
Mus musculus ?
-
?

Synonyms

Synonyms Comment Organism
ClpP Protease
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Mus musculus