Atypical BSE: Current Knowledge and Knowledge Gaps

Dudas, Sandor; Czub, Stefanie

doi:10.14252/foodsafetyfscj.2016028

Cited by 15 publications

(14 citation statements)

References 36 publications

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“…However, since the three Holstein cattle carrying the K211 somatic mutation in the medulla oblongata were not investigated as to whether K211 somatic mutation could be observed in other brain regions, it is difficult to conclude that this somatic mutation was only found in the medulla oblongata. In addition, since atypical BSE has shown to be a prominent brain pathology in the frontal cortex [ 17 , 18 ], if the atypical BSE was caused by a somatic mutation of the PRNP gene, it is expected that the somatic mutation was also found in the cerebral cortex. Thus, further investigation of the K211 somatic mutation is highly desirable in other brain regions, including the cerebral cortex of the three Holstein cattle carrying K211 somatic mutation in the medulla oblongata.…”

Section: Discussionmentioning

confidence: 99%

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First Report of the Potential Bovine Spongiform Encephalopathy (BSE)-Related Somatic Mutation E211K of the Prion Protein Gene (PRNP) in Cattle

Won

Kim

Jeong

2020

IJMS

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Bovine spongiform encephalopathy (BSE) is a prion disease characterized by spongiform degeneration and astrocytosis in the brain. Unlike classical BSE, which is caused by prion-disease-contaminated meat and bone meal, the cause of atypical BSE has not been determined. Since previous studies have reported that the somatic mutation in the human prion protein gene (PRNP) has been linked to human prion disease, the somatic mutation of the PRNP gene was presumed to be one cause of prion disease. However, to the best of our knowledge, the somatic mutation of this gene in cattle has not been investigated to date. We investigated somatic mutations in a total of 58 samples, including peripheral blood; brain tissue including the medulla oblongata, cerebellum, cortex, and thalamus; and skin tissue in 20 individuals from each breed using pyrosequencing. In addition, we estimated the deleterious effect of the K211 somatic mutation on bovine prion protein by in silico evaluation tools, including PolyPhen-2 and PANTHER. We found a high rate of K211 somatic mutations of the bovine PRNP gene in the medulla oblongata of three Holsteins (10% ± 4.4%, 28% ± 2%, and 19.55% ± 3.1%). In addition, in silico programs showed that the K211 somatic mutation was damaging. To the best of our knowledge, this study is the first to investigate K211 somatic mutations of the bovine PRNP gene that are associated with potential BSE progression.

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Section: Discussionmentioning

confidence: 99%

“…However, novel forms of BSE and atypical BSEs have been reported in many countries. Unlike classical BSE, the cause of atypical BSE has not been determined [ 16 , 17 , 18 ].…”

Section: Introductionmentioning

confidence: 99%

First Report of the Potential Bovine Spongiform Encephalopathy (BSE)-Related Somatic Mutation E211K of the Prion Protein Gene (PRNP) in Cattle

Won

Kim

Jeong

2020

IJMS

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“…In addition to this finding, atypical BSE cases have been reported in countries such as Norway, Romania, and Brazil, where no C-BSE cases were reported 80) . Therefore, the occurrence of H-BSE and L-BSE was unlikely due to feed-mediated infection, but is likely due to sporadic nature 17,81) L-BSE can be readily transmitted to various animals by experimental infection. The experiments using bovine-PrP overexpressing Tg (Tgbov XV) mice, human-PrP overexpressing Tg mice, macaques, and cattle have suggested that the virulence of L-BSE (BASE) is higher than that of C-BSE 18,[82][83][84] .…”

Section: Transmission Characteristics Of Atypical Bsementioning

confidence: 99%

Bovine Spongiform Encephalopathy – A Review from the Perspective of Food Safety

2019

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Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease that belongs to transmissible spongiform encephalopathy (TSE). Since the first case was identified in the UK in 1986, BSE spread to other countries including Japan. Its incidence peaked in 1992 in the UK and from 2001 to 2006 in many other countries, but a feed ban aimed at eliminating the recycling of the BSE agent and other control measures aimed at preventing food and feed contamination with the agent were highly effective at reducing the spread of BSE. In 2004, two types of atypical BSE, H-type BSE (H-BSE) and L-type BSE (L-BSE), which differ from classical BSE (C-BSE), were found in France and Italy. Atypical BSE, which is assumed to occur spontaneously, has also been detected among cattle in other countries including Japan. The BSE agent including atypical BSE agent is a unique food-safety hazard with different chemical and biological properties from the microbial pathogens and toxic chemicals that contaminate food. In this review, we summarize the reported findings on the tissue distribution of BSE prions in infected cattle and other aspects of BSE, as well as the control measures against the disease employed in Japan. Topics that require further studies are discussed based on the summarized findings from the perspective of food safety. Key word: BSE, atypical, vCJD, control measure, origin tified in the UK in 1986 1). The UK epidemic was attributed to the exposure to an infectious agent in 1981-1982 in association with a dramatic reduction in the use of organic solvents in the manufacture of meat and bone meal (MBM) 2). The annual number of BSE cases reported in the UK increased

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“…They also differ from Norwegian reindeer CWD and from CWD strains in North America [ 12 , 13 ]. They are thought to represent sporadic prion disease as has been described in other species, rather than contagious CWD [ 14 – 16 ]. In 2020, a new case of contagious CWD was discovered in a wild reindeer on the Hardanger mountain plateau south of Nordfjella [ 17 ], representing a new geographical region for infection.…”

Section: Introductionmentioning

confidence: 99%

No evidence of uptake or propagation of reindeer CWD prions in environmentally exposed sheep

et al. 2022

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Background Chronic wasting disease (CWD) is a prion disease of cervids first reported in North America in the 1960s. In Europe, CWD was first diagnosed in 2016 in a wild reindeer in Norway. Detection of two more cases in the same mountain area led to the complete culling of this partially confined reindeer population of about 2400 animals. A total of 19 CWD positive animals were identified. The affected area is extensively used for the grazing of sheep during summers. There are many mineral licks intended for sheep in the area, but these have also been used by reindeer. This overlap in area use raised concerns for cross-species prion transmission between reindeer and sheep. In this study, we have used global positioning system (GPS) data from sheep and reindeer, including tracking one of the CWD positive reindeer, to investigate spatial and time-relevant overlaps between these two species. Since prions can accumulate in lymphoid follicles following oral uptake, samples of gut-associated lymphoid tissue (GALT) from 425 lambs and 78 adult sheep, which had grazed in the region during the relevant timeframe, were analyzed for the presence of prions. The recto-anal mucosa associated lymphoid tissue (RAMALT) from all the animals were examined by histology, immunohistochemistry (IHC) and enzyme-linked immunosorbent assay (ELISA), and the ileal Peyer's patch (IPP) from a subsample of 37 lambs were examined by histology and IHC, for the detection of prions. Results GPS data showed an overlap in area use between the infected reindeer herd and the sheep. In addition, the GPS positions of an infected reindeer and some of the sampled sheep showed temporospatial overlap. No prions were detected in the GALT of the investigated sheep even though the mean lymphoid follicle number in RAMALT and IPP samples were high. Conclusion The absence of prions in the GALT of sheep that have shared pasture with CWD-infected reindeer, may suggest that transmission of this novel CWD strain to sheep does not easily occur under the conditions found in these mountains. We document that the lymphoid follicle rich RAMALT could be a useful tool to screen for prions in sheep.

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Atypical BSE: Current Knowledge and Knowledge Gaps

Cited by 15 publications

References 36 publications

First Report of the Potential Bovine Spongiform Encephalopathy (BSE)-Related Somatic Mutation E211K of the Prion Protein Gene (PRNP) in Cattle

First Report of the Potential Bovine Spongiform Encephalopathy (BSE)-Related Somatic Mutation E211K of the Prion Protein Gene (PRNP) in Cattle

Bovine Spongiform Encephalopathy – A Review from the Perspective of Food Safety

No evidence of uptake or propagation of reindeer CWD prions in environmentally exposed sheep

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