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Huntington's disease and Group I metabotropic glutamate receptors

Fabiola M Ribeiro1, Rita G W Pires, Stephen S G Ferguson

  • 1Departamento de Bioquimica e Imunologia, ICB, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil.

Molecular Neurobiology
|December 15, 2010
PubMed

Insights

Huntington's disease (HD) involves mutant huntingtin protein altering cell signaling. Understanding its effect on glutamate receptors is key to developing new HD therapies.

Area of Science:

  • Neuroscience
  • Genetics
  • Molecular Biology

Background:

  • Huntington's disease (HD) is an inherited neurodegenerative disorder caused by a polyglutamine expansion in the huntingtin (htt) protein.
  • Mutant htt disrupts cell signaling pathways, affecting neurotransmitter and trophic factor systems, leading to neuronal death in the striatum and cortex.

Purpose of the Study:

  • To review recent findings on how mutant huntingtin protein affects cell signaling, particularly glutamatergic pathways.
  • To clarify the role of metabotropic glutamate receptors (mGluR1/5) in neuronal cell death in HD.

Main Methods:

  • Review of existing literature on Huntington's disease, huntingtin protein, cell signaling, and glutamate receptors.
  • Analysis of studies investigating the interaction between mutant htt and neurotransmitter systems, focusing on mGluR1/5.

Main Results:

  • Mutant htt causes alterations in dopaminergic, glutamatergic, and cannabinoid signaling pathways.
  • The precise role of mGluR1/5 activation in neuronal cell death in HD remains controversial, with evidence suggesting both protective and detrimental effects.

Conclusions:

  • Understanding the impact of mutant htt on glutamatergic signaling is crucial for elucidating HD pathogenesis.
  • Targeting glutamate receptor signaling presents a potential therapeutic strategy for Huntington's disease.

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