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Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
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Amyloid Fibrils03:03

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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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Kuru: the first prion disease.

Paweł P Liberski1, Beata Sikorska, Paul Brown

  • 1Department of Molecular Pathology and Neuropathology, Medical University Lodz, Lodz, Poland. ppliber@csk.am.lodz.pl

Advances in Experimental Medicine and Biology
|March 14, 2012
PubMed
Summary

Kuru disease, a fatal neurodegenerative condition, was transmitted to chimpanzees, classifying it as a transmissible spongiform encephalopathy (TSE). This research linked ritualistic cannibalism to the spread of this human prion disease.

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Area of Science:

  • Neuroscience
  • Pathology
  • Epidemiology

Background:

  • Kuru disease, reported in Papua New Guinea, is a fatal neurodegenerative disorder.
  • The Fore people's language defines "kuru" as shivering from fever and cold.
  • Ritualistic cannibalism was identified as the transmission route.

Observation:

  • D. Carleton Gajdusek demonstrated kuru's transmissibility to chimpanzees.
  • Kuru was classified as a transmissible spongiform encephalopathy (TSE), also known as slow unconventional virus disease.
  • The disease presents as invariably fatal cerebellar ataxia with tremors and involuntary movements.

Findings:

  • Kuru is neuropathologically characterized by the presence of amyloid "kuru" plaques.
  • The study established a link between ritualistic cannibalism and the transmission of kuru.
  • Gajdusek's work classified kuru as a TSE, a significant advancement in understanding prion diseases.

Implications:

  • This research advanced the understanding of human prion diseases and their transmission mechanisms.
  • The classification of kuru as a TSE opened new avenues for research into neurodegenerative disorders.
  • Understanding the epidemiological factors of kuru provided insights into disease spread within isolated populations.