Diffuse deposition of immunohistochemically labeled prion protein in the granular layer of the cerebellum in a patient with Creutzfeldt-Jakob disease

H. A. Kretzschmar, T. Kitamoto, J. Doerr-Schott, P. Mehraein, J. Tateishi

Research output: Contribution to journalArticlepeer-review

21 Citations (Scopus)

Abstract

Amyloid plaques in Creutzfeldt-Jakob discase, kuru, and Gerstmann-Sträussler-Scheinker syndrome are known to contain an abnormal isoform of a cellular protein, the prion protein (PrP). The prion protein in its normal cellular isoform is a membranebound glycoprotein of unknown function. The mechanisms causing a modification of PrP and accumulation in amyloid plaques are unknown. Here we present a case of Creutzfeldt-Jakob disease with widespread deposition of immunohistochemically labeled PrP in the internal granular layer of the cerebellum. Immunohistochemically labeled PrP was deposited in delicate granules, which often were associated with cellular processes or the cytoplams of undefined cells, or diffusely deposited in the neuropil.

Original languageEnglish
Pages (from-to)536-540
Number of pages5
JournalActa neuropathologica
Volume82
Issue number6
DOIs
Publication statusPublished - 1991 Nov 1
Externally publishedYes

Keywords

  • Creutzfeldt-Jakob disease
  • Intracellular accumulation
  • Prion protein

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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