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Biphasic developmental changes in Ca2+/calmodulin-dependent proteins in R6/2 Huntington's disease mice
A Wallace Deckel1, Robert Elder, Greg Fuhrer
1Department of Psychiatry, University of Connecticut Health Center, 263 Farmington Avenue, 06030-2103, USA.
Neuroreport
|April 26, 2002
Summary
Huntington's disease (HD) disrupts calcium-dependent proteins. In HD mice, calmodulin kinase IV and neuronal nitric oxide synthase (NOS) expression initially increased then decreased, impacting brain function.
Area of Science:
- Neuroscience
- Molecular Biology
- Genetics
Background:
- Huntington's disease (HD) is characterized by widespread disturbances in calcium-dependent proteins.
- These protein alterations are observed in both human patients and HD mouse models.
Purpose of the Study:
- To investigate the expression patterns of calmodulin kinase IV and neuronal nitric oxide synthase (NOS) in an HD mouse model.
- To understand the impact of these protein expression changes on the striatum throughout the disease progression.
Main Methods:
- Utilized the Bates R6/2 transgenic mouse model for Huntington's disease.
- Analyzed the expression levels of calmodulin kinase IV and neuronal nitric oxide synthase (NOS) at different ages (3, 6, and 11 weeks).
Main Results:
- Presymptomatic 3-week-old HD mice showed increased expression of calmodulin kinase IV and neuronal NOS in the striatum and cortex.
- Expression levels of these proteins decreased at 6 weeks (early symptom onset) and 11 weeks (advanced disease).
Conclusions:
- Mutant Huntington's disease protein may disrupt striatal function by altering the regulation of calmodulin kinase IV and neuronal NOS expression.
- These dynamic changes in protein expression suggest a complex role in HD pathogenesis.
- The findings highlight potential molecular pathways affected by mutant HD protein impacting neuronal function.