Hypersensitivity to antigen derived from brain tissue in patients with Huntington's disease and in individuals at risk.

Walls, Ronald S.; Ruwoldt, A.

doi:10.1172/jci111397

Cited by 6 publications

(1 citation statement)

References 10 publications

(8 reference statements)

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“…(Husby, Li et al 1977) Another Journal of Clinical Investigation paper found that there is hypersensitivity to antigens derived from brain tissue of patients with Huntington's disease. (Walls and Ruwoldt 1984) In a Science paper, cellular immunity mediator: migration inhibitory factor is also up-regulated in Huntington's patients. (Barkley, Hardiwidjaja et al 1977) MIF can play an important role in TH17 immunity.…”

Section: Discussionmentioning

confidence: 99%

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

2011

Nat Prec

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Huntington's disease is an inherited neurodegenerative disorder. It belongs to the polyQ accumulation disease with mutant Huntingtin protein. The mutant Huntingtin has expended CAG triplet repeats which make it easily 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 is Treg cells from thymus to prevent autoimmune reaction against the mutant Huntingtin. When the patient grows older, the accumulated mutant Huntingtin 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 via activating apoptotic cascade. Once we know the pathogenesis of Huntington's disease, we can use immunology knowledge to develop preventive or therapeutic strategies to cope with this detrimental disorder.

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Section: Discussionmentioning

confidence: 99%

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

2011

Nat Prec

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The interaction of a huntington disease factor with receptors for the neurotoxin kainic acid

et al. 1991

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A factor from mammalian and human brain, which inhibits the rate of migration of leukocytes obtained from sufferers from Huntington disease (Walls and Ruwoldt, 1984), inhibited the specific binding of the neurotoxin [3H]kainic acid to rat brain synaptic membranes. The factor was present in sucrose-particulate but not in soluble fractions from rat sub-cortical tissue, and was destroyed by tryptic digestion. Whereas an ammonium sulfate fraction of direct saline extracts of brain (Walls and Ruwoldt, 1984) gave poor chromatography on HPLC, prior separation of a sucrose-particulate fraction resulted in much improved chromatography. There was a good concordance between leukocyte migration inhibitory activity and [3H]kainic acid binding inhibitory activity. The factor may be an endogenous modulator of the kainic acid subset of receptors for the excitatory neurotransmitter glutamic acid.

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Material prepared from normal brain inhibits migration of leukocytes from Huntington's disease patients

Walls

Ruwoldt

Hurst

1986

Journal of the Neurological Sciences

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Hypersensitivity to antigen derived from brain tissue in patients with Huntington's disease and in individuals at risk.

Cited by 6 publications

References 10 publications

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

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

The interaction of a huntington disease factor with receptors for the neurotoxin kainic acid

Material prepared from normal brain inhibits migration of leukocytes from Huntington's disease patients

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