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Published on: March 11, 2020
The corticostriatal pathway in Huntington's disease
Carlos Cepeda1, Nanping Wu, Véronique M André
1Mental Retardation Research Center, David Geffen School of Medicine, University of California at Los Angeles, Los Angeles, CA 90095, USA.
Progress in Neurobiology
|December 16, 2006
Summary
Huntington's disease (HD) involves corticostriatal pathway dysfunction, affecting brain circuits before cell loss. Therapies must target specific brain regions and disease stages for HD treatment.
Area of Science:
- Neuroscience
- Genetics
- Neurology
Background:
- The corticostriatal pathway is crucial for striatal function and Huntington's disease (HD) pathogenesis.
- Alterations in this pathway precede significant neuronal loss in HD models.
Purpose of the Study:
- To review findings from genetic mouse models of HD regarding corticostriatal pathway alterations.
- To understand the role of circuit dysfunctions in HD phenotype development.
Main Methods:
- Review of genetic Huntington's disease mouse models.
- Analysis of morphological and functional changes in corticostriatal circuits.
Main Results:
- Corticostriatal pathway dysfunctions and cortical microcircuit changes occur early in HD development.
- Striatal medium-spiny neuron intrinsic properties and NMDA receptor sensitivity are altered.
- These circuit changes interact with disease progression and age.
Conclusions:
- Neuronal circuits, both cortical and striatal, are central to HD phenotype expression.
- Therapeutic strategies for HD require region-specific and temporally-aware interventions targeting these circuits.
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