Distribution of Abnormal Prion Protein in a Sheep affected with L-type Bovine Spongiform Encephalopathy

Matsuura, Yuichi; Iwamaru, Yoshifumi; Masujin, Kentaro; Imamura, Morikazu; Mohri, Satoshi; Yokoyama, Takashi; Okada, Hiroyuki

doi:10.1016/j.jcpa.2012.11.231

Cited by 16 publications

(23 citation statements)

References 28 publications

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“…In addition we have observed an increased reactivity with the N-terminal antibody which is not reported in other ovine L-BSE transmissions [40, 41]. This particular characteristic, normally associated with classical scrapie, is also observed on transmission of classical BSE to sheep but seen as a more gradual N-terminal antibody detection that increases after serial subpassage [56].…”

Section: Discussionsupporting

confidence: 57%

“…Previous reports on the transmission of L-BSE to sheep have only described the inoculation of animals that are ARQ/ARQ [40, 41] or ARQ/ARR [41]. This present study extends the range of sheep genotypes inoculated experimentally, to include the VRQ, and AFRQ haplotypes.…”

Section: Discussionmentioning

confidence: 84%

“…These include cattle [36–39], sheep [40–42] and this report, lemurs [43], macaques [23, 24], hamsters [25, 44, 45] and transgenic mice overexpressing bovine, ovine or human PrP [20, 25–27, 39, 42, 44, 46–48]. In contrast to classical BSE, L-BSE does not transmit to wild type [20] Spiropoulos and Simmons, unpublished data] or transgenic mice that overexpress murine PrP [45, 47], although exceptions have been reported [20].…”

Section: Discussionmentioning

confidence: 99%

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L-BSE experimentally transmitted to sheep presents as a unique disease phenotype

Simmons¹,

Chaplin²,

Konold

et al. 2016

Vet Res

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Apart from prion protein genotype, the factors determining the host range and susceptiblity for specific transmissible spongiform encephalopathy agents remain unclear. It is known that bovine atypical L-BSE can transmit to a range of species including primates and humanised transgenic mice. It is important, therefore, that there is as broad an understanding as possible of how such isolates might present in food animal species and how robust they are on inter- and intra-species transmission to inform surveillance sytems and risk assessments. This paper demonstrates that L-BSE can be intracerebrally transmitted to sheep of several genotypes, with the exception of ARR/ARR animals. Positive animals mostly present with a cataplectic form of disease characterized by collapsing episodes and reduced muscle tone. PrP accumulation is confined to the nervous system, with the exception of one animal with lymphoreticular involvement. In Western blot there was maintenance of the low molecular mass and glycoform profile associated with L-BSE, irrespective of ovine host genotype, but there was a substantially higher N-terminal antibody signal relative to the core-specific antibody, which is similar to the ratio associated with classical scrapie. The disease phenotype was maintained on experimental subpassage, but with a shortened survival time indicative of an original species barrier and subsequent adaptation. Passive surveillance approaches would be unlikely to identify such cases as TSE suspects, but current statutory active screening methods would be capable of detecting such cases and classifying them as unusual and requiring further investigation if they were to occur in the field.Electronic supplementary materialThe online version of this article (doi:10.1186/s13567-016-0394-1) contains supplementary material, which is available to authorized users.

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Section: Discussionsupporting

confidence: 57%

Section: Discussionmentioning

confidence: 84%

Section: Discussionmentioning

confidence: 99%

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L-BSE experimentally transmitted to sheep presents as a unique disease phenotype

Simmons¹,

Chaplin²,

Konold

et al. 2016

Vet Res

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“…As with BSE, the identification of L-type BSE in cattle raised the issue of what the diagnostic criteria would be in small ruminants if they are susceptible, and experimental transmission studies were designed to characterise the phenotype of the disease in sheep [90][91][92] .…”

Section: L-type Bsementioning

confidence: 99%

Pathology of Animal Transmissible Spongiform Encephalopathies (TSEs)

Spiropoulos

Simmons

2017

Food Safety

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Pathology is the study of the structural and functional changes produced by diseases or − more specifically − the lesions they cause. To achieve this pathologists employ various approaches. These include description of lesions that are visible to the naked eye which are the subject of anatomic pathology and changes at the cellular level that are visible under the microscope, the subject of histopathology. Changes at the molecular level which are identified by probes that target specific molecules − mainly proteins that are detected using immunohistochemistry (IHC). As transmissible spongiform encephalopathies (TSEs) do not cause visible lesions anatomic pathology is not applicable to their study. For decades the application of histopathology to detect vacuoles or plaques was the only means of confirming TSE disease. The subsequent discovery of the cellular prion protein (PrP C ) and its pathogenic isoform, PrP Sc , which is a ubiquitous marker of TSEs, led to the production of anti-PrP antibodies, and enabled the development of PrP Sc detection techniques such as immunohistochemistry, Histoblot and PETblot that have evolved in parallel with similar biochemical methods such as Western blot and ELISA. These methods offer greater sensitivity than histopathology in TSE diagnosis and crucially they can be applied to analyze various phenotypic aspects of single TSE sources increasing the amount of data and offering higher discriminatory power. The above principles are applied to diagnose and define TSE phenotypes which form the basis of strain characterisation.

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“…al. reported phenotypic similarities between L-BSE and bovine passaged TME derived from the Wisconsin outbreak [11]. We previously reported that sheep are susceptible to bovine adapted TME (bTME) after intracranial inoculation [12], and the resulting disease phenotype is similar to L-BSE in sheep with PrP Sc not detected in lymphoid tissues [13,14]. These similarities prompted an investigation to compare ovine passaged bovine TME (o-bTME) with other TSEs from cattle, C-BSE and L-BSE.…”

Section: Introductionmentioning

confidence: 98%

Bovine adapted transmissible mink encephalopathy is similar to L-BSE after passage through sheep with the VRQ/VRQ genotype but not VRQ/ARQ

Cassmann

Moore

Kokemuller

et al. 2020

Preprint

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Background Transmissible mink encephalopathy (TME) is a fatal neurologic disease of farmed mink. Evidence indicates that TME and L-BSE are similar and may be linked in some outbreaks of TME. We previously transmitted bovine adapted TME (bTME) to sheep. The present study compared ovine passaged bTME (o-bTME) to C-BSE and L-BSE in transgenic mice expressing wild type bovine prion protein (TgBovXV). To directly compare the transmission efficiency of all prion strains in this study, we considered the attack rates and mean incubation periods. Additional methods for strain comparison were utilized including lesion profiles, fibril stability, and western blotting. Results Sheep donor genotype elicited variable disease phenotypes in bovinized mice. Inoculum derived from a sheep with the VRQ/VRQ genotype (o-bTMEVV) resulted in an attack rate, incubation period, western blot profile, and neuropathology most similar to bTME and L-BSE. Conversely, donor material from a sheep with the VRQ/ARQ genotype (o-bTMEAV) elicited a phenotype distinct from o-bTMEVV, bTME and L-BSE. The TSE with the highest transmission efficiency in bovinized mice was L-BSE. The tendency to efficiently transmit to TgBovXV mice decreased in the order bTME, C-BSE, o-bTMEVV, and o-bTMEAV. The transmission efficiency of L-BSE was approximately 1.3 times higher than o-bTMEVV and 3.2 times higher than o-bTMEAV. Conclusions Our findings provide insight on how sheep host genotype modulates strain genesis and influences interspecies transmission characteristics. Given that the transmission efficiencies of L-BSE and bTME are higher than C-BSE, coupled with previous reports of L-BSE transmission to mice expressing the human prion protein, continued monitoring for atypical BSE is advisable in order to prevent occurrences of interspecies transmission that may affect humans or other species.

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Distribution of Abnormal Prion Protein in a Sheep affected with L-type Bovine Spongiform Encephalopathy

Cited by 16 publications

References 28 publications

L-BSE experimentally transmitted to sheep presents as a unique disease phenotype

L-BSE experimentally transmitted to sheep presents as a unique disease phenotype

Pathology of Animal Transmissible Spongiform Encephalopathies (TSEs)

Bovine adapted transmissible mink encephalopathy is similar to L-BSE after passage through sheep with the VRQ/VRQ genotype but not VRQ/ARQ

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