Oral contamination with bovine spongiform encephalopathy (BSE) agent in susceptible PRNP genotype sheep results in widespread distribution of prion in the host. Because ARR homozygous sheep are considered to be resistant to transmissible spongiform encephalopathies, they have been selected to eradicate scrapie from sheep flocks and to protect the human food chain from small ruminant BSE risk. However, results presented here show that several months after an oral challenge with BSE agent, healthy ARR/ARR sheep can accumulate significant amounts of PrP(Sc) in the spleen.
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http://dx.doi.org/10.1099/vir.0.81318-0 | DOI Listing |
Animals (Basel)
November 2024
Korea Zoonosis Research Institute, Jeonbuk National University, 820-120 Hana-ro, Iksan 54531, Republic of Korea.
Prion diseases are fatal neurodegenerative disorders caused by the misfolding of the normal cellular prion protein (PrP) into its infectious isoform (PrP). Although prion diseases in humans, sheep, goats, and cattle have been extensively studied, feline spongiform encephalopathy (FSE) remains poorly understood. Genetic factors, particularly polymorphisms in the prion protein gene () and protein gene (), have been linked to prion disease susceptibility in various species.
View Article and Find Full Text PDFThis report presents the results of surveillance on transmissible spongiform encephalopathies in cattle, sheep, goats, cervids and other species, and genotyping in sheep and goats, carried out in 2023 by 27 Member States (MS, EU27), the United Kingdom (in respect of Northern Ireland, (XI)) and other eight non-EU reporting countries: Bosnia and Herzegovina, Iceland, Montenegro, North Macedonia, Norway, Serbia, Switzerland (the data reported by Switzerland include those of Liechtenstein) and Türkiye. In total, 948,165 cattle were tested by EU27 and XI (-3%, compared with 2022), with five atypical BSE cases reported (four H-type: two in Spain, one in France and one in Ireland; one L-type in the Netherlands); and 46,096 cattle by eight non-EU reporting countries with two atypical BSE cases reported by Switzerland. Three additional atypical BSE cases were reported by UK (1), USA (1) and Brazil (1).
View Article and Find Full Text PDFCureus
November 2024
Acute Medicine, Peterborough City Hospital, Peterborough, GBR.
Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder that leads to rapid cognitive decline, dementia, and neurological deterioration. CJD has several forms, including sporadic CJD (sCJD), which accounts for most cases, and variant CJD (vCJD), linked to exposure to bovine spongiform encephalopathy (BSE or mad cow disease). The disease is caused by abnormal prion proteins, which damage the brain and lead to death.
View Article and Find Full Text PDFMicrobiol Immunol
November 2024
Department of Biochemistry and Cell Biology, National Institute of Infectious Diseases, Shinjuku-ku, Tokyo, Japan.
Bovine spongiform encephalopathy (BSE) is a prion disease in cattle caused by classical-type (C-), L-type (L-), or H-type (H-) BSE prions. While C-BSE prions are zoonotic agents responsible for variant Creutzfeldt-Jakob disease, L- and H-BSE prions are believed not to be connected to human prion diseases. However, L-BSE prions have been shown to transmit to cynomolgus monkeys (Macaca fascicularis), suggesting they may have zoonotic potential.
View Article and Find Full Text PDFPLoS One
October 2024
Department of Microbiology, Immunology and Pathology, Prion Research Center, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, Colorado, United States of America.
Zoonotic transmission of bovine spongiform encephalopathy or mad cow disease, by presumed consumption of infected beef, has increased awareness of the public health risk associated with prion diseases. Chronic wasting disease (CWD) affects moose, elk, and deer, all of which are frequently consumed by humans. Clear evidence of CWD transmission to humans has not been demonstrated, yet, establishing whether CWD prions are present in muscle tissue preferentially consumed by humans is of increasing interest.
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