ArticleNeuro-degenerative diseases2011
Impaired brain creatine kinase activity in Huntington's disease.
Article in Neuro-degenerative diseases, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
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Who cites it
24 citing papers in PubMed, 48 citations in OpenAlex.
- The Role of MicroRNAs in Neurodegeneration: Insights from Huntington's Disease.Molecular neurobiology · 2025Review
- Simultaneous monitoring of glycogen, creatine, and phosphocreatine in type II glycogen storage disease using saturation transfer MRI.Magnetic resonance in medicine · 2025Article
- Dietary Marine Oils Selectively Decrease Obesogenic Diet-Derived Carbonylation in Proteins Involved in ATP Homeostasis and Glutamate Metabolism in the Rat Cerebellum.Antioxidants (Basel, Switzerland) · 2024Article
- Induced pluripotent stem cell-derived and directly reprogrammed neurons to study neurodegenerative diseases: The impact of aging signatures.Frontiers in aging neuroscience · 2022Review
- Altered high-energy phosphate and membrane metabolism in Pelizaeus-Merzbacher disease using phosphorus magnetic resonance spectroscopy.Brain communications · 2022Article
- Biomarkers Utility: At the Borderline between Cardiology and Neurology.Journal of cardiovascular development and disease · 2021Review
- The Therapeutic Potential of Neuronal K-Cl Co-Transporter KCC2 in Huntington's Disease and Its Comorbidities.International journal of molecular sciences · 2020Review
- Mitochondrial Creatine Kinase is Decreased in the Serum of Idiopathic Parkinson's Disease Patients.Aging and disease · 2019Article
- Insights into GABAOpen biology · 2018Review
- Article
- Towards an Understanding of Energy Impairment in Huntington's Disease Brain.Journal of Huntington's disease · 2017Review
- Quantitative Proteomic Analysis Reveals Similarities between Huntington's Disease (HD) and Huntington's Disease-Like 2 (HDL2) Human Brains.Journal of proteome research · 2016Article
- N-Acetylcysteine improves mitochondrial function and ameliorates behavioral deficits in the R6/1 mouse model of Huntington's disease.Translational psychiatry · 2015Article
- Assessment of changes in energy metabolism parameters provoked by carbon tetrachloride in Wistar rats and the protective effect of white grape juice.Toxicology reports · 2015Article
- Metabolism in HD: still a relevant mechanism?Movement disorders : official journal of the Movement Disorder Society · 2014Review
- PGC-1α, mitochondrial dysfunction, and Huntington's disease.Free radical biology & medicine · 2013Review
- Role of oxidative DNA damage in mitochondrial dysfunction and Huntington's disease pathogenesis.Free radical biology & medicine · 2013Review
- Energy dysfunction in Huntington's disease: insights from PGC-1α, AMPK, and CKB.Cellular and molecular life sciences : CMLS · 2012Review
- Mitochondrial dysfunction in neurodegenerative diseases.The Journal of pharmacology and experimental therapeutics · 2012Review
- R6/2 Huntington's disease mice develop early and progressive abnormal brain metabolism and seizures.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2012Article
Corrections and comments
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
Abstract
backgroundHuntington's disease (HD) is associated with impaired energy metabolism in the brain. Creatine kinase (CK) catalyzes ATP-dependent phosphorylation of creatine (Cr) into phosphocreatine (PCr), thereby serving as readily available high-capacity spatial and temporal ATP buffering.
objectiveSubstantial evidence supports a specific role of the Cr/PCr system in neurodegenerative diseases. In the brain, the Cr/PCr ATP-buffering system is established by a concerted operation of the brain-specific cytosolic enzyme BB-CK and ubiquitous mitochondrial uMt-CK. It is not yet established whether the activity of these CK isoenzymes is impaired in HD.
methodsWe measured PCr, Cr, ATP and ADP in brain extracts of 3 mouse models of HD - R6/2 mice, N171-82Q and HdhQ(111) mice - and the activity of CK in cytosolic and mitochondrial brain fractions from the same mice.
resultsThe PCr was significantly increased in mouse HD brain extracts as compared to nontransgenic littermates. We also found an approximately 27% decrease in CK activity in both cytosolic and mitochondrial fractions of R6/2 and N171-82Q mice, and an approximately 25% decrease in the mitochondria from HdhQ(111) mice. Moreover, uMt-CK and BB-CK activities were approximately 63% lower in HD human brain samples as compared to nondiseased controls.
conclusionOur findings lend strong support to the role of impaired energy metabolism in HD, and point out the potential importance of impairment of the CK-catalyzed ATP-buffering system in the etiology of HD.
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