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Literature summary extracted from

  • Lin, Y.S.; Cheng, T.H.; Chang, C.P.; Chen, H.M.; Chern, Y.
    Enhancement of brain-type creatine kinase activity ameliorates neuronal deficits in Huntingtons disease (2013), Biochim. Biophys. Acta, 1832, 742-753.
    View publication on PubMed

Application

EC Number Application Comment Organism
2.7.3.2 medicine strategies aimed at increasing isozyme CKB expression might lead to the development of therapeutic treatments for Huntington's disease Homo sapiens

Cloned(Commentary)

EC Number Cloned (Comment) Organism
2.7.3.2 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

EC Number Localization Comment Organism GeneOntology No. Textmining
2.7.3.2 cytosol
-
 Mus musculus 5829
-

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
2.7.3.2 Mg2+ required Homo sapiens
2.7.3.2 Mg2+ required Mus musculus

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
2.7.3.2 ATP + creatine Homo sapiens
-
 ADP + phosphocreatine
-
 r
2.7.3.2 ATP + creatine Mus musculus
-
 ADP + phosphocreatine
-
 r
2.7.3.2 ATP + creatine Mus musculus R6/2
-
 ADP + phosphocreatine
-
 r

Organism

EC Number Organism UniProt Comment Textmining
2.7.3.2 Homo sapiens P12277 gene Ckb
-
2.7.3.2 Mus musculus Q04447 67 R6/2 mice and 31 wild-type B6CBAFI/J mice, gene Ckb
-
2.7.3.2 Mus musculus R6/2 Q04447 67 R6/2 mice and 31 wild-type B6CBAFI/J mice, gene Ckb
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
2.7.3.2 brain
-
 Homo sapiens
-
2.7.3.2 brain
-
 Mus musculus
-
2.7.3.2 neuron primary cortical neurons from mutant R6/2 mice and wild-type B6CBAFI/J mice Mus musculus
-
2.7.3.2 striatum
-
 Mus musculus
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2.7.3.2 ATP + creatine
-
 Homo sapiens ADP + phosphocreatine
-
 r
2.7.3.2 ATP + creatine
-
 Mus musculus ADP + phosphocreatine
-
 r
2.7.3.2 ATP + creatine
-
 Mus musculus R6/2 ADP + phosphocreatine
-
 r

Synonyms

EC Number Synonyms Comment Organism
2.7.3.2 brain-type creatine kinase
-
 Homo sapiens
2.7.3.2 brain-type creatine kinase
-
 Mus musculus
2.7.3.2 ckb
-
 Homo sapiens
2.7.3.2 ckb
-
 Mus musculus

Cofactor

EC Number Cofactor Comment Organism Structure
2.7.3.2 ADP
-
 Homo sapiens
2.7.3.2 ADP
-
 Mus musculus
2.7.3.2 ATP
-
 Homo sapiens
2.7.3.2 ATP
-
 Mus musculus

Expression

EC Number Organism Comment Expression
2.7.3.2 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

EC Number General Information Comment Organism
2.7.3.2 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
2.7.3.2 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
2.7.3.2 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
2.7.3.2 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