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Literature summary for 2.7.3.2 extracted from
- Enhancement of brain-type creatine kinase activity ameliorates neuronal deficits in Huntingtons disease (2013), Biochim. Biophys. Acta, 1832, 742-753.
Application
| Application | Comment | Organism |
|---|---|---|
| medicine | strategies aimed at increasing isozyme CKB expression might lead to the development of therapeutic treatments for Huntington's disease | Homo sapiens |
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
| gene Ckb, real-time quantitative reverse-transcription enzyme expression analysis. Compared to Neuro-2a cells expressing a control construct, expression of mHTT (109Q) significantly inhibits CKB promoter activity | Mus musculus |
Localization
| Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|
| cytosol | - |
Mus musculus | 5829 | - |
Metals/Ions
| Metals/Ions | Comment | Organism | Structure |
|---|---|---|---|
| Mg2+ | required | Homo sapiens |  |
| Mg2+ | required | Mus musculus | |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| ATP + creatine | Homo sapiens | - |
ADP + phosphocreatine | - |
r | |
| ATP + creatine | Mus musculus | - |
ADP + phosphocreatine | - |
r | |
| ATP + creatine | Mus musculus R6/2 | - |
ADP + phosphocreatine | - |
r | |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | P12277 | gene Ckb | - |
| Mus musculus | Q04447 | 67 R6/2 mice and 31 wild-type B6CBAFI/J mice, gene Ckb | - |
| Mus musculus R6/2 | Q04447 | 67 R6/2 mice and 31 wild-type B6CBAFI/J mice, gene Ckb | - |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| brain | - |
Homo sapiens | - |
| brain | - |
Mus musculus | - |
| neuron | primary cortical neurons from mutant R6/2 mice and wild-type B6CBAFI/J mice | Mus musculus | - |
| striatum | - |
Mus musculus | - |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| ATP + creatine | - |
Homo sapiens | ADP + phosphocreatine | - |
r | |
| ATP + creatine | - |
Mus musculus | ADP + phosphocreatine | - |
r | |
| ATP + creatine | - |
Mus musculus R6/2 | ADP + phosphocreatine | - |
r | |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| brain-type creatine kinase | - |
Homo sapiens |
| brain-type creatine kinase | - |
Mus musculus |
| ckb | - |
Homo sapiens |
| ckb | - |
Mus musculus |
Cofactor
| Cofactor | Comment | Organism | Structure |
|---|---|---|---|
| ADP | - |
Homo sapiens | |
| ADP | - |
Mus musculus | |
| ATP | - |
Homo sapiens | |
| ATP | - |
Mus musculus | |
Expression
| Organism | Comment | Expression |
|---|---|---|
| Mus musculus | mutant mHTT (109Q) significantly suppresses the activity of the promoter of the CKB gene, which contributes to the lowered CKB expression in Huntington's disease. Exogenous expression of wild-type CKB, but not a dominant negative CKB mutant, rescues the phenotype | down |
General Information
| General Information | Comment | Organism |
|---|---|---|
| malfunction | downregulation of brain-type creatine kinase in brain of a Huntington's disease mouse model. Huntington's disease is a hereditary neurodegenerative disorder caused by a CAG repeat expansion in the huntingtin (HTT) gene. Mutant HTT (mHTT) suppresses the activity of the CKB gene promoter, which contributes to the lowered CKB expression in Huntington's disease. Exogenous expression of wild-type CKB, but not a dominant negative CKB mutant, rescues the ATP depletion, aggregate formation, impaired proteasome activity, and shortened neurites induced by mHTT. Negative regulation of isozyme CKB by mHTT is a key event in the pathogenesis of Huntington's disease and contributes to the neuronal dysfunction associated with Huntington's disease | Mus musculus |
| malfunction | downregulation of brain-type creatine kinase in brains of Huntington's disease patients. Huntington's disease is a hereditary neurodegenerative disorder caused by a CAG repeat expansion in the huntingtin (HTT) gene | Homo sapiens |
| physiological function | brain-type creatine kinase is an enzyme involved in energy homeostasis via the phosphocreatine-creatine kinase system, the CK system plays a critical role in energy homeostasis and ATP distribution | Homo sapiens |
| physiological function | brain-type creatine kinase is an enzyme involved in energy homeostasis via the phosphocreatine-creatine kinase system, the CK system plays a critical role in energy homeostasis and ATP distribution. Creatine kinase activity is critical for the beneficial effects of isozyme CKB of enhancing proteasome activity and reducing aggregate formation | Mus musculus |
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