EBioMedicine (Oct 2016)

Prion-Seeding Activity Is widely Distributed in Tissues of Sporadic Creutzfeldt-Jakob Disease Patients

  • Hanae Takatsuki, PhD,
  • Takayuki Fuse, PhD,
  • Takehiro Nakagaki, MD, PhD,
  • Tsuyoshi Mori, PhD,
  • Ban Mihara, MD, PhD,
  • Masaki Takao, MD, PhD,
  • Yasushi Iwasaki, MD, PhD,
  • Mari Yoshida, MD, PhD,
  • Shigeo Murayama, MD, PhD,
  • Ryuichiro Atarashi, MD, PhD,
  • Noriyuki Nishida, MD, PhD,
  • Katsuya Satoh, MD, PhD

DOI
https://doi.org/10.1016/j.ebiom.2016.08.033
Journal volume & issue
Vol. 12, no. C
pp. 150 – 155

Abstract

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Human prion diseases are neurodegenerative disorders caused by abnormally folded prion proteins in the central nervous system. These proteins can be detected using the quaking-induced conversion assay. Compared with other bioassays, this assay is extremely sensitive and was used in the present study to determine prion distribution in sporadic Creutzfeldt-Jakob disease patients at autopsy. Although infectivity of the sporadic form is thought to be restricted within the central nervous system, results showed that prion-seeding activities reach 106/g from a 50% seeding dose in non-neuronal tissues, suggesting that prion-seeding activity exists in non-neural organs, and we suggested that non-neural tissues of 106/g SD50 did not exist the infectivity.

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