Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits

Okada, Hiroyuki; Iwamaru, Yoshifumi; Imamura, Morikazu; Masujin, Kentaro; Matsuura, Yuichi; Shimizu, Yoshihisa; Kasai, Kazuo; Mohri, Satoshi; Yokoyama, Takashi; Czub, Stefanie

doi:10.1186/1297-9716-42-79

Cited by 45 publications

(107 citation statements)

References 56 publications

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“…The brain tissue and cerebrospinal fluid (CSF) samples from cattle experimentally inoculated intracerebrally with either the C-, L-, or H-BSE prion strain were obtained from the National Institute of Animal Health (NIAH), National Agriculture and Food Research Organization (NARO), Japan (25,43,44 Western blot analysis of PrP Res from C-, L-, or H-BSE-affected cattle. Brain tissue (cortex) samples from uninfected, C-, L-, or H-BSEaffected cattle were homogenized (20% concentration [wt/vol]) in phosphate-buffered saline (PBS) using a multibead shocker (Yasui Kikai).…”

Section: Methodsmentioning

confidence: 99%

Detection of Atypical H-Type Bovine Spongiform Encephalopathy and Discrimination of Bovine Prion Strains by Real-Time Quaking-Induced Conversion

et al. 2016

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Many mammalian species can be afflicted with prion diseases or transmissible spongiform encephalopathies. Prion diseases are neurodegenerative, transmissible, untreatable, and fatal (reviewed in references 1 and 2). The underlying pathogenesis of prion diseases involves the conversion of the host's normal prion protein, PrP C , to abnormal forms that are usually more protease resistant, multimeric, and insoluble. The multimeric and insoluble forms have been generically called PrP Sc (PrP-scrapie), or more functionally PrP Res (protease resistant) and PrP D (disease associated). At least some of these forms comprise the transmissible agent or prion (3-10). Multiple prion strains can be propagated within a given host species, giving consistently different clinical, pathological, and molecular phenotypes (11-16).The first prion disease to be recognized in cattle was classical bovine spongiform encephalopathy (C-BSE). C-BSE was likely caused primarily by widespread prion contamination of cattle feed (17). After peaking in the early 1990s, the incidence of C-BSE has now been greatly reduced by regulatory measures that limit its horizontal spread. C-BSE is the only known zoonotic prion disease, having caused variant Creutzfeldt-Jakob disease (vCJD) in humans who presumably consumed contaminated beef. Although new clinical cases of vCJD are rare (http://www.cjd.ed.ac .uk/documents/figs.pdf), a recent survey of appendices in the United Kingdom suggests a high incidence of subclinical vCJD infections, at ϳ1:2,000 of the population born between 1941 and 1985 (18).Since the C-BSE epidemic, two atypical strains of BSE, H-type BSE (H-BSE) and L-type BSE (L-BSE), have also been identified in cattle. PrPRes is usually composed of a mixture of glycosylated and unglycosylated molecules, and the various BSE strains can be differentiated biochemically using Western blotting of postmortem brain tissue samples by comparing the proteinase K (PK)-treated PrPRes banding patterns (19)(20)(21)(22). The H and L types of BSE are classified by their respective high and low apparent molecular masses of the unglycosylated PrP Res band. These atypical BSE strains, which are rare (Ͻ100 cases identified worldwide), tend to affect older animals (23) and appear to represent sporadic forms of bovine prion diseases (24). Despite the apparent rarity of the various types of BSE, the facts that H-and L-BSE appear to arise spontaneously and have distinct transmissibilities (20, 25-32) make it important to be able to detect and differentiate them to reduce the risk of transmission to cattle or other species, such as humans.Several in vitro methods have been developed to detect C-, L-, and H-BSE. Commercially available rapid immunochemical tests for PrP D can, in the best cases, give positive responses from 10 Ϫ3 to 10 Ϫ4 dilutions of postmortem brain tissues with high levels of PrP D (33, 34). Immunoblotting for PrP Res can detect BSE-infected tissues with similar sensitivity and also discriminate between the bovine strains based on the relative electro...

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Section: Methodsmentioning

confidence: 99%

Detection of Atypical H-Type Bovine Spongiform Encephalopathy and Discrimination of Bovine Prion Strains by Real-Time Quaking-Induced Conversion

et al. 2016

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“…16 Because no field case of H-BSE has been found to date in Japan, we utilized a cow experimentally infected with the H-BSE prion by intracranial administration (experimental code number: 9458). 44 Tissues positive for the H-BSE prion were from the brain stem. The DNA sequence of the PrP coding region of this cow was identical to that of Bos taurus PrP in the database (accession number: AJ298878).…”

Section: Brain Tissuesmentioning

confidence: 99%

Evaluation of rapid post-mortem test kits for bovine spongiform encephalopathy (BSE) screening in Japan: Their analytical sensitivity to atypical BSE prions

Hagiwara

Iwamaru

Tabeta

et al. 2017

Prion

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ABSTRACT. A classical type of bovine spongiform encephalopathy (C-BSE), recognized in 1987, had a large impact on public health due to its zoonotic link to variant Creutzfeldt-Jakob disease by the human consumption of dietary products contaminated with the C-BSE prion. Thus, a number of countries implemented BSE surveillance using rapid post-mortem test kits that were approved for detection of the C-BSE prion in the cattle brain. However, as atypical BSE (L-and H-BSE) cases emerged in subsequent years, the efficacy of the kits for the detection of atypical BSE prions became a matter of concern. In response to this, laboratories in the European Union and Canada evaluated the kits used in their countries. Here, we carried out an evaluation study of NippiBL Ò , a kit currently used for BSE screening in Japan. By applying the kit to cattle brains of field cases of C-BSE and L-BSE, and an experimental case of H-BSE, we showed its comparable sensitivities to C, L-, and H-BSE prions, and satisfactory performance required by the European Food Safety Authority. In addition to NippiBL Ò , two kits (TeSeE Ò and FRELISA Ò ) formerly used in Japan were effective for detection of the L-BSE prion, although the two kits were unable to be tested for the H-BSE prion due to the discontinuation of domestic sales during this study. These results indicate that BSE screening Correspondence to: Ken'ichi Hagiwara;

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“…If atypical BSE does transmit with a longer incubation, it would contradict the observations in the intracranial challenge. Cattle challenged intra-cranially with atypical BSE have a documented incubation time of 14-21 months [29][30][31] . Intracranial challenge of cattle with classical BSE results in significantly longer incubation with clinical disease starting around 24 months 12) .…”

Section: Atypical Bse Transmissionmentioning

confidence: 99%

Atypical BSE: Current Knowledge and Knowledge Gaps

Dudas

Czub

2017

Food Safety

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Atypical BSE is an invariably fatal neurologic disease of cattle caused by misfolded prion proteins with different conformations than those associated with classical BSE. Evidence suggests that these atypical BSE types are sporadic or genetic prion diseases of cattle and the relevance of these diseases, as far as natural transmissibility, is still unknown. Different misfolded prion protein conformations also result in unique biochemical characteristics. This raised concerns about detection of atypical BSE on rapid test platforms designed and validated for classical BSE prions. Despite the differences in the misfolded prion protein characteristics, studies have shown that the tests also work well for detecting the known types of atypical BSE. A new question that has recently emerged is related to the possibility of additional forms of atypical BSE. Initially reactive bovine brain samples on certain rapid surveillance tests have sparked debate about the true BSE status of these samples. Work is currently underway to determine if these samples are infectious and if they eventually result in neurologic disease in cattle. Results of these studies could impact future BSE diagnostic testing programs as well as human and animal health policies.Key words: atypical BSE, bovine spongiform encephalopathy, neurologic disease, prion, PrP Atypical BSEBovine spongiform encephalopathy (BSE) is a prion disease of cattle that is invariably fatal and zoonotic. Three type of BSE have been detected in a number of countries around the world. Classical BSE is the most common types of BSE and the cause of the BSE epidemic in the United Kingdom 1) . In 2004, two additional types of BSE were identified mostly in older animals 2,3) and were named according to the electrophoretic migration of their protease resistant prion protein or PrP RES . High or H type BSE PrP RES is slightly larger, migrates slower and is detected in a position higher than classical BSE, while low or L type BSE PrP RES is smaller, migrates faster and is detected in a lower position than classical BSE during electrophoresis. Along with these differences in size, H and L type atypical BSE also have different biochemical and transmission characteristics, PrP RES distribution and pose different challenges for detection and diagnosis.

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Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits

Cited by 45 publications

References 56 publications

Detection of Atypical H-Type Bovine Spongiform Encephalopathy and Discrimination of Bovine Prion Strains by Real-Time Quaking-Induced Conversion

Detection of Atypical H-Type Bovine Spongiform Encephalopathy and Discrimination of Bovine Prion Strains by Real-Time Quaking-Induced Conversion

Evaluation of rapid post-mortem test kits for bovine spongiform encephalopathy (BSE) screening in Japan: Their analytical sensitivity to atypical BSE prions

Atypical BSE: Current Knowledge and Knowledge Gaps

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