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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. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...

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Detection of Abnormal Prion Protein by Immunohistochemistry
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Detection of Abnormal Prion Protein by Immunohistochemistry

Published on: May 5, 2023

Visual pathology in animal prion diseases.

X Ye1

  • 1Department of Ophthalmology, The Mount Sinai Medical Center, New York, NY 10029-6547, USA. Xuemin.ye@mssm.edu

Histology and Histopathology
|October 2, 2009
PubMed
Summary

Prion diseases, or transmissible spongiform encephalopathies, affect the central nervous system and visual pathways in humans and animals. This review examines prion infectivity and visual pathology in affected species.

Area of Science:

  • Neuroscience
  • Veterinary Medicine
  • Ophthalmology

Background:

  • Prion diseases, known as transmissible spongiform encephalopathies (TSEs), are progressive neurodegenerative disorders affecting humans and animals.
  • These diseases are transmissible across species and can lead to severe central nervous system damage, including microglia activation, astrocytosis, and neuronal loss.

Purpose of the Study:

  • To review the prion agent and its infectivity within the eye and brain's visual system.
  • To discuss visual and oculomotor pathology observed in animal prion diseases.

Main Methods:

  • Review of existing literature on prion diseases and their impact on the visual system.
  • Analysis of pathological findings in the retina, optic nerve, and visual cortex of affected animals.

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Main Results:

  • Prion diseases target the eye and brain's visual pathways, affecting retinal layers, optic nerve, and visual cortex.
  • Pathological changes are documented in various animal models, including sheep, deer, and experimental infections.

Conclusions:

  • Prion diseases cause significant visual and oculomotor dysfunction due to central nervous system and ocular pathology.
  • Further discussion will explore the influence of PrP genotypes and PrPSc types on these disorders.