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Related Concept Videos

Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

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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Determining Immune System Suppression versus CNS Protection for Pharmacological Interventions in Autoimmune Demyelination
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Huntington's Disease: An Immune Perspective.

Annapurna Nayak1, Rafia Ansar, Sunil K Verma

  • 1Centre for Infection, Immunity and Disease Mechanisms, Biosciences School of Health Sciences and Social Care, Brunel University, West London UB8 3PH, UK.

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|August 31, 2011
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Summary

Huntington's disease (HD) involves neuroinflammation, driven by mutant huntingtin protein and aberrant immune responses. This review summarizes the immunological aspects contributing to HD's development and progression.

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Area of Science:

  • Neuroscience
  • Immunology
  • Genetics

Background:

  • Huntington's disease (HD) is a progressive neurodegenerative disorder.
  • It is characterized by the abnormal expansion of CAG trinucleotide repeats.
  • Neuroinflammation is a common feature in neurodegenerative diseases, including HD, leading to brain pathology.

Purpose of the Study:

  • To summarize the immunological features associated with the development and progression of Huntington's disease.
  • To explore the role of immune activation in the HD nervous system, an area requiring further research.

Main Methods:

  • Literature review of immunological aspects in Huntington's disease.
  • Synthesis of current understanding regarding neuroinflammation and immune response in HD pathogenesis.

Main Results:

  • Aberrant immune responses and neuroinflammation are implicated in HD neurodegeneration.
  • Aggregated mutant huntingtin protein triggers pathological immune reactions in the HD nervous system.

Conclusions:

  • The immunological landscape of Huntington's disease is crucial for understanding its progression.
  • Further research into immune activation in HD is warranted to uncover potential therapeutic targets.