Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients

Atsushi Kobayashi, Kunimasa Arima, Masafumi Ogawa, Miho Murata, Takahiro Fukuda, Tetsuyuki Kitamoto

Research output: Contribution to journalArticlepeer-review

19 Citations (Scopus)

Abstract

Plaque-type deposition of prion protein (PrP) in the brain has been extremely rare in sporadic Creutzfeldt-Jakob disease patients with methionine homozygosity at polymorphic codon 129 of the PrP gene and type 1 abnormal isoform of PrP (sCJD-MM1). Here we report three sCJD-MM1 patients who showed prominent PrP-positive amyloid plaques in the cerebral and cerebellar white matter. All three patients showed clinical courses of long duration (2 years ≤), particularly at the end-stage. The white matter of these patients was severely damaged because of the prolonged disease duration. Furthermore, Alzheimer's amyloid precursor protein, which accumulates within the axonal swellings under pathological conditions, co-accumulated with the PrP-amyloid plaques. These findings suggest that the axonal damage reflecting the prolonged disease duration causes the deposition of PrP-amyloid plaques in the white matter. The present study shows that PrP-amyloid plaques can occur in the white matter of sCJD-MM1 cases.

Original languageEnglish
Pages (from-to)561-566
Number of pages6
JournalActa neuropathologica
Volume116
Issue number5
DOIs
Publication statusPublished - 2008

Keywords

  • Amyloid plaque
  • Creutzfeldt-Jakob disease
  • Prion protein
  • White matter

ASJC Scopus subject areas

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

Fingerprint Dive into the research topics of 'Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients'. Together they form a unique fingerprint.

Cite this