Cited by 196Prion Diseases and Protein MisfoldingAlzheimer's disease research and treatmentsAmyloidosis: Diagnosis, Treatment, Outcomes

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Neuropathology of prion diseases

Budka, Herbert·2003·British Medical Bulletin

DOI 10.1093/bmb/66.1.121

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Abstract

In prion diseases, neuropathology has remained the most important tool to give a definite diagnosis, and neuropathological research has contributed significantly to our current pathogenetic understanding. Immunohistochemistry for the disease-associated prion protein (PrP(Sc)) is indispensable for the neuropathological confirmation of prion diseases. The amount and distribution of PrP(Sc) deposits do not always correlate with type and severity of local tissue damage. PrP(Sc) deposition occurs only where neuronal parenchyma is present; in scarred infarctions with prominent gliosis, PrP(Sc) does not accumulate. Early, severe and selective loss affects a subset of inhibitory GABAergic neurons both in human and experimental prion diseases. The central pathogenetic cascade includes oxidative stress to neurons and their apoptosis. New patterns of PrP(Sc) immunoreactivity include granular ganglionic and tiny adaxonal PrP(Sc) deposits in peripheral nervous tissue, and dendritic cells and macrophages in vessel walls, suggesting that mobile haematogenous cells may be involved in spread of prions.

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Authors: Budka, H.
Affiliations: University of Vienna (AT)
Corresponding author: Budka, Herbert
ORCID: Budka, Herbert
Venue: British Medical Bulletin
Publisher: Oxford University Press (OUP)
Published: 2003-06-01
Volume: 66
Issue: 1
Pages: 121-130
ISSN: 0007-1420 (ISSN-L)
1471-8391
DOI: 10.1093/bmb/66.1.121
OpenAlex: W2150554290
Cited by: 196
References: 56
Citations by year: 2026: 3 · 2025: 9 · 2024: 1 · 2023: 5 · 2022: 9 · 2021: 4 · 2020: 9 · 2019: 7 · 2018: 4 · 2017: 8 · 2016: 10 · 2015: 10 · 2014: 9 · 2013: 9 · 2012: 10
FWCI: 0.98
Type: journal article
Language: en
Open access: closed
Locations: British Medical Bulletin (publishedVersion)
PubMed (publishedVersion)
Funders: Bundesministerium für Gesundheit und Frauen
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
Health Sciences › Medicine › Internal Medicine › Amyloidosis: Diagnosis, Treatment, Outcomes
Keywords: prion disease, neuropathology, oxidative stress, neuronal apoptosis, dendritic cells, macrophages, pathogenesis
MeSH terms: Animals, Brain, Humans, Immunohistochemistry, Neurons, Prions, Species Specificity, Prion Diseases, Peripheral Nervous System

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