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

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.

• Publication year

  2016

• Venue

  EBioMedicine

• Publication date

  2016-08-24

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1016/j.ebiom.2016.08.033PMID 27612591PMCID 5078574
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Tissue-based map of the human proteome

  2015cited by this paper
• Rapid and Quantitative Assay of Amyloid-Seeding Activity in Human Brains Affected with Prion Diseases

  2015cited by this paper
• The Genotype-Tissue Expression (GTEx) project

  2013cited by this paper
• Re-Assessment of PrPSc Distribution in Sporadic and Variant CJD

  2013cited by this paper
• Ultrasensitive human prion detection in cerebrospinal fluid by real-time quaking-induced conversion

  2011cited by this paper
• Rapid End-Point Quantitation of Prion Seeding Activity with Sensitivity Comparable to Bioassays

  2010cited by this paper
• Kuru in the 21st century--an acquired human prion disease with very long incubation periods.

  2006cited by this paper
• Kuru in the 21st century-an acquired human prion disease with very long incubation periods

  2006cited by this paper
• PrPTSE Distribution in a Primate Model of Variant, Sporadic, and Iatrogenic Creutzfeldt-Jakob Disease

  2005cited by this paper
• Humanized knock-in mice expressing chimeric prion protein showed varied susceptibility to different human prions.

  2003cited by this paper
• Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease.

  2003cited by this paper
• Creutzfeldt-Jakob disease probably acquired from a cadaveric dura mater graft. Case report.

  1988cited by this paper
• Serological response to the hepatitis delta virus in hepatitis D.

  1987cited by this paper
• Evidence for case-to-case transmission of Creutzfeldt-Jakob disease

  1982cited by this paper
• Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery.

  1977cited by this paper

• Creutzfeldt-Jakob Disease with Abnormality of Periaqueductal Gray Matter Region at Diffusion-Weighted Magnetic Resonance Imaging.

  2025cites this paper
• Sodium hypochlorite inactivation of human CJD prions

  2024cites this paper
• CSF biomarkers for prion diseases.

  2022cites this paper
• Laboratory Diagnosis of Creutzfeldt-Jakob Disease.

  2022cites this paper
• The risk of Creutzfeldt–Jakob disease infection in cadaveric surgical training

  2022cites this paper
• Wide distribution of prion infectivity in the peripheral tissues of vCJD and sCJD patients

  2021cites this paper
• Abnormal prion protein deposits with high seeding activities in the skeletal muscle, femoral nerve, and scalp of an autopsied case of sporadic Creutzfeldt–Jakob disease

  2021cites this paper
• Harnessing the Physiological Functions of Cellular Prion Protein in the Kidneys: Applications for Treating Renal Diseases

  2021cites this paper
• Formalin RT-QuIC assay detects prion-seeding activity in formalin-fixed brain samples from sporadic Creutzfeldt-Jakob disease patients.

  2021cites this paper
• Development of α-Synuclein Real-Time Quaking-Induced Conversion as a Diagnostic Method for α-Synucleinopathies

  2021cites this paper
• Correlation between Bioassay and Protein Misfolding Cyclic Amplification for Variant Creutzfeldt-Jakob Disease Decontamination Studies

  2020cites this paper
• Detection of Pathognomonic Biomarker PrPSc and the Contribution of Cell Free-Amplification Techniques to the Diagnosis of Prion Diseases

  2020cites this paper
• Administration of FK506 from Late Stage of Disease Prolongs Survival of Human Prion-Inoculated Mice

  2020cites this paper
• Improving the Predictive Value of Prion Inactivation Validation Methods to Minimize the Risks of Iatrogenic Transmission With Medical Instruments

  2020cites this paper
• Evidence for sporadic Creutzfeldt-Jakob disease being an acquired disease

  2019cites this paper
• Prion-related peripheral neuropathy in sporadic Creutzfeldt-Jakob disease

  2018cites this paper
• How can we increase the number of autopsies for prion diseases? A model system in Japan.

  2017cites this paper
• Prion Diagnosis: Application of Real-Time Quaking-Induced Conversion

  2017cites this paper
• Prion seeding activity and infectivity in skin samples from patients with sporadic Creutzfeldt-Jakob disease

  2017cites this paper
• Absence of Evidence for a Causal Link between Bovine Spongiform Encephalopathy Strain Variant L-BSE and Known Forms of Sporadic Creutzfeldt-Jakob Disease in Human PrP Transgenic Mice

  2016cites this paper
• Zur Aufbereitung von Medizin­ pro dukten unter besonderer Berücksichtigung der Creutzfeldt­ Jakob­Krankheit und ihrer Variante: Eine Betrachtung 20 Jahre nach dem Bericht der deutschen Task Force vCJK

  year unknowncites this paper