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Huntington’s disease is a TH17 related autoimmune disorder against mutant Huntingtin coded by multiple CAG triplets

Abstract

Huntington’s disease is an inherited neurodegenerative disorder. It belongs to the polyQ accumulation disease with mutant Huntington protein. The mutant Huntington has expanded CAG triplet repeats which make it easy to accumulate. However, the exact mechanism causing Huntington’s disease pathophysiology is unknown. Here, I propose that Huntington’s disease is an autoimmune disease. During childhood, there are Treg cells from the thymus to prevent autoimmune reaction against mutant Huntington. When the patient grows older with thymus atrophy, the accumulated mutant Huntington triggers TH17 related inflammatory reaction via heat shock proteins and toll-like receptor activation. Then, TH17 related cytokines such as TNFα, IL1, and IL6 cause neuron degeneration by activating apoptotic cascade. Once we know the pathogenesis of Huntington’s disease, we can use immunological knowledge to develop preventive or therapeutic strategies to cope with this detrimental disorder.

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Correspondence to Wanchung(Wanjiung) Hu.

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Hu, W. Huntington’s disease is a TH17 related autoimmune disorder against mutant Huntingtin coded by multiple CAG triplets. Nat Prec (2011). https://doi.org/10.1038/npre.2011.6129.1

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Keywords

  • Huntington's disease
  • TH17
  • CAG repeats
  • polyglutamine
  • Huntingtin
  • Autoimmune

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