scholarly journals Prion infectivity in the spleen of a PRNP heterozygous individual with subclinical variant Creutzfeldt–Jakob disease

Brain ◽  
2013 ◽  
Vol 136 (4) ◽  
pp. 1139-1145 ◽  
Author(s):  
Matthew T. Bishop ◽  
Abigail B. Diack ◽  
Diane L. Ritchie ◽  
James W. Ironside ◽  
Robert G. Will ◽  
...  
Keyword(s):  
Heterozygous Individual ◽  
Prion Infectivity ◽  
Jakob Disease

scholarly journals Lack of Prion Infectivity in Fixed Heart Tissue from Patients with Creutzfeldt-Jakob Disease or Amyloid Heart Disease

2013 ◽  
Vol 87 (17) ◽  
pp. 9501-9510 ◽  
Author(s):  
S. A. Priola ◽  
A. E. Ward ◽  
S. A. McCall ◽  
M. Trifilo ◽  
Y. P. Choi ◽  
...  
Keyword(s):  
Heart Disease ◽  
Heart Tissue ◽  
Amyloid Heart Disease ◽  
Prion Infectivity ◽  
Jakob Disease

scholarly journals Wide distribution of prion infectivity in the peripheral tissues of vCJD and sCJD patients

2021 ◽  
Vol 141 (3) ◽  
pp. 383-397
Author(s):  
Jean-Yves Douet ◽  
Alvina Huor ◽  
Hervé Cassard ◽  
Séverine Lugan ◽  
Naima Aron ◽  
...  
Keyword(s):  
Prion Disease ◽  
Wide Distribution ◽  
Human Prion Disease ◽  
Marked Contrast ◽  
Peripheral Tissues ◽  
Spontaneous Formation ◽  
Prion Infectivity ◽  
Clinical Procedures ◽  
Jakob Disease ◽  
The Central Nervous System

AbstractSporadic Creutzfeldt-Jakob disease (sCJD) is the commonest human prion disease, occurring most likely as the consequence of spontaneous formation of abnormal prion protein in the central nervous system (CNS). Variant Creutzfeldt–Jakob disease (vCJD) is an acquired prion disease that was first identified in 1996. In marked contrast to vCJD, previous investigations in sCJD revealed either inconsistent levels or an absence of PrPSc in peripheral tissues. These findings contributed to the consensus that risks of transmitting sCJD as a consequence of non-CNS invasive clinical procedures were low. In this study, we systematically measured prion infectivity levels in CNS and peripheral tissues collected from vCJD and sCJD patients. Unexpectedly, prion infectivity was detected in a wide variety of peripheral tissues in sCJD cases. Although the sCJD infectivity levels varied unpredictably in the tissues sampled and between patients, these findings could impact on our perception of the possible transmission risks associated with sCJD.

scholarly journals Assessing Prion Infectivity of Human Urine in Sporadic Creutzfeldt-Jakob Disease

2012 ◽  
Vol 18 (1) ◽  
pp. 21-28 ◽  
Author(s):  
Silvio Notari ◽  
Liuting Qing ◽  
Maurizio Pocchiari ◽  
Ayuna Dagdanova ◽  
Kristin Hatcher ◽  
...  
Keyword(s):  
Human Urine ◽  
Prion Infectivity ◽  
Jakob Disease

scholarly journals Vaporized Hydrogen Peroxide and Ozone Gas Synergistically Reduce Prion Infectivity on Stainless Steel Wire

2021 ◽  
Vol 22 (6) ◽  
pp. 3268
Author(s):  
Hideyuki Hara ◽  
Junji Chida ◽  
Agriani Dini Pasiana ◽  
Keiji Uchiyama ◽  
Yutaka Kikuchi ◽  
...  
Keyword(s):  
Hydrogen Peroxide ◽  
Stainless Steel ◽  
Prion Diseases ◽  
Steel Wire ◽  
Mouse Bioassay ◽  
Infectious Agents ◽  
Prion Infectivity ◽  
Steel Wires ◽  
Medical Instruments ◽  
Jakob Disease

Prions are infectious agents causing prion diseases, which include Creutzfeldt–Jakob disease (CJD) in humans. Several cases have been reported to be transmitted through medical instruments that were used for preclinical CJD patients, raising public health concerns on iatrogenic transmissions of the disease. Since preclinical CJD patients are currently difficult to identify, medical instruments need to be adequately sterilized so as not to transmit the disease. In this study, we investigated the sterilizing activity of two oxidizing agents, ozone gas and vaporized hydrogen peroxide, against prions fixed on stainless steel wires using a mouse bioassay. Mice intracerebrally implanted with prion-contaminated stainless steel wires treated with ozone gas or vaporized hydrogen peroxide developed prion disease later than those implanted with control prion-contaminated stainless steel wires, indicating that ozone gas and vaporized hydrogen peroxide could reduce prion infectivity on wires. Incubation times were further elongated in mice implanted with prion-contaminated stainless steel wires treated with ozone gas-mixed vaporized hydrogen peroxide, indicating that ozone gas mixed with vaporized hydrogen peroxide reduces prions on these wires more potently than ozone gas or vaporized hydrogen peroxide. These results suggest that ozone gas mixed with vaporized hydrogen peroxide might be more useful for prion sterilization than ozone gas or vaporized hydrogen peroxide alone.

scholarly journals Experimental inoculation of CD11c+ B1 lymphocytes, CD68+ macrophages, or platelet-rich plasma from scrapie-infected sheep into susceptible sheep results in variable infectivity

2020 ◽  
Vol 2 (9) ◽  
Author(s):  
Najiba Mammadova ◽  
Eric D. Cassmann ◽  
S. Jo Moore ◽  
Eric M. Nicholson ◽  
Justin J. Greenlee
Keyword(s):  
Disease Transmission ◽  
Platelet Rich Plasma ◽  
White Blood Cells ◽  
Transmissible Spongiform Encephalopathies ◽  
Blood Components ◽  
Spongiform Encephalopathies ◽  
Prion Infection ◽  
Prion Infectivity ◽  
Jakob Disease ◽  
The Central Nervous System

Many studies have demonstrated prion infectivity in whole blood and blood components in a variety of transmissible spongiform encephalopathies of livestock and rodents, and variant Creutzfeldt–Jakob disease in humans, as well as an association between pathogenic prion protein (PrPSc) and different immune cells (e.g. follicular dendritic cells, T and B lymphocytes, monocytes and tingible body macrophages). To further investigate the role of various blood components in prion disease transmission, we intracranially inoculated genetically susceptible VRQ/ARQ and ARQ/ARQ sheep with inocula composed of CD11c+ B1 lymphocytes, CD68 +macrophages, or platelet-rich plasma derived from clinically ill sheep infected with the US no. 13–7 scrapie agent. At the completion of the study, we found that VRQ/ARQ and ARQ/ARQ sheep inoculated with CD11c+ B1 lymphocytes and CD68+ macrophages developed scrapie with detectable levels of PrPSc in the central nervous system and lymphoreticular system, while those inoculated with platelet-rich plasma did not develop disease and did not have detectable PrPSc by immunohistochemistry or enzyme immunoassay. This study complements and expands on earlier findings that white blood cells harbour prion infectivity, and reports CD11c+ B1 lymphocytes and CD68+ macrophages as additional targets for possible preclinical detection of prion infection in blood.

scholarly journals Prion infectivity in variant Creutzfeldt-Jakob disease rectum

Gut ◽  
2007 ◽  
Vol 56 (1) ◽  
pp. 90-94 ◽  
Author(s):  
J D F Wadsworth ◽  
S Joiner ◽  
K Fox ◽  
J M Linehan ◽  
M Desbruslais ◽  
...  
Keyword(s):  
Prion Infectivity ◽  
Jakob Disease

Upbeat nystagmus as the initial clinical sign of Creutzfeldt-Jakob disease

2005 ◽  
Vol 32 (S 4) ◽  
Author(s):  
M Strupp ◽  
V.C Zingler ◽  
K Jahn ◽  
M Glaser ◽  
H Kretzschmar ◽  
...  
Keyword(s):  
Clinical Sign ◽  
Jakob Disease ◽  
Upbeat Nystagmus
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