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Related Concept Videos

Parkinson Disease ll: Pathophysiology01:24

Parkinson Disease ll: Pathophysiology

Parkinson disease (PD) is a progressive neurodegenerative disorder primarily affecting movement, with additional non-motor features. Its pathophysiology involves complex interactions among genetic susceptibility, environmental exposures, and cellular dysfunction, including dopaminergic neuron loss, protein aggregation, and mitochondrial impairment.Selective NeurodegenerationA key feature is the degeneration of dopaminergic neurons in the substantia nigra pars compacta, leading to reduced...
Huntington Disease l: Introduction01:21

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Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...

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Synaptic Dysfunction in Huntington's Disease: Lessons from Genetic Animal Models.

Carlos Cepeda1, Michael S Levine1

  • 1IDDRC, Jane and Terry Semel Institute for Neuroscience & Human Behavior, David Geffen School of Medicine, University of California, Los Angeles, CA, USA.

The Neuroscientist : a Review Journal Bringing Neurobiology, Neurology and Psychiatry
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Summary

Huntington

Keywords:
Huntington’s diseasebasal gangliadopaminegenetic modelsglutamatestriatumsynaptic activity

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Area of Science:

  • Neuroscience
  • Neurodegenerative Diseases
  • Genetics

Background:

  • Huntington's disease (HD) involves progressive brain degeneration.
  • Genetic animal models have advanced understanding of HD's functional and structural brain changes.
  • Research is expanding beyond the striatum to the entire cortico-basal ganglia-cortical loop.

Purpose of the Study:

  • To review and analyze current research on synaptic alterations in the brain of rodent models of Huntington's disease.
  • To explore the role of cortical maldevelopment and mutant huntingtin protein interactions in synaptic dysfunction.
  • To examine the progressive disconnection in the corticostriatal pathway and its impact on neuronal function and degeneration.

Main Methods:

  • Review and analysis of existing studies on Huntington's disease rodent models.
  • Focus on synaptic alterations in the cerebral cortex and basal ganglia.
  • Examination of changes in glutamate and dopamine release in the striatum.

Main Results:

  • Cortical maldevelopment contributes to corticostriatal pathway synaptic dysfunction.
  • Mutant huntingtin protein interactions with synaptic proteins are implicated.
  • Progressive corticostriatal disconnection engages extrasynaptic NMDA receptors, leading to cell degeneration.
  • Biphasic changes in striatal glutamate and dopamine release correlate with disease stages.

Conclusions:

  • Synaptic dysfunction in Huntington's disease is complex, involving cortical and striatal alterations.
  • Understanding these synaptic changes offers insights into disease mechanisms and symptomatology.
  • Therapeutic strategies targeting synaptic dysfunction are being explored.