Co-existence of classical scrapie and Nor98 in a sheep from an Italian outbreak

Mazza, Maria; Iulini, Barbara; Vaccari, Gabriele; Acutis, Pier Luigi; Martucci, Francesca; Esposito, Elena; Peletto, Simone; Barocci, Simone; Chiappini, Barbara; Corona, Cristiano; Barbieri, Ilaria; Caramelli, Maria; Agrimi, Umberto; Casalone, Cristina; Nonno, Romolo

doi:10.1016/j.rvsc.2009.11.015

Cited by 37 publications

(36 citation statements)

References 48 publications

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“…Natural mixed atypical/classical infection of single sheep has been reported elsewhere (Mazza et al, 2010). Intriguingly, recent PrP Sc molecular studies have found evidence of natural mixed infections including CH1641-like strains in sheep (Langeveld et al, 2014) and experimental inoculation studies have suggested that the phenotype of atypical scrapie can in some instances change into that of CH1641 during passage in sheep (Simmons et al, 2015).…”

Section: Discussionmentioning

confidence: 90%

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Archival search for historical atypical scrapie in sheep reveals evidence for mixed infections

Chong

Kennedy

Goldmann

et al. 2015

Journal of General Virology

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Section: Discussionmentioning

confidence: 90%

“…VRQ/AL 141 RQ and AL 141 RQ/AL 141 RQ (Fediaevsky et al, 2008). Indeed, there is evidence for both scrapie forms occurring in a single AL 141 RQ/ AF 141 RQ animal (Mazza et al, 2010).…”

Section: Introductionmentioning

confidence: 99%

Archival search for historical atypical scrapie in sheep reveals evidence for mixed infections

Chong

Kennedy

Goldmann

et al. 2015

Journal of General Virology

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“…Analyses of brain tissue collected from two healthy cattle and nine Italian BSE-infected field cases (Table 1), four classified as C type and five as L type, were done by Western blotting on 150-mg (Ϯ20 mg) equivalents of each sample as described previously (37). Briefly, each sample was separated by SDS-PAGE on 14% polyacrylamide handmade minigels and then transferred onto polyvinylidene difluoride (PVDF) membranes (Immobilon P; Millipore, Billerica, MA) for 2 h at 60 V. The membranes were blocked with 5% bovine serum albumin (BSA) in Tris-buffered saline (TBS) for 1 h and incubated at 4°C overnight with monoclonal antibody 6H4 (0.9 g/ml, epitope 144-152, DYEDRYYRE; Prionics, Schlieren-Zurich, Switzerland).…”

Section: Methodsmentioning

confidence: 99%

Detection and Discrimination of Classical and Atypical L-Type Bovine Spongiform Encephalopathy by Real-Time Quaking-Induced Conversion

et al. 2015

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Statutory surveillance of bovine spongiform encephalopathy (BSE) indicates that cattle are susceptible to both classical BSE (C-BSE) and atypical forms of BSE. Atypical forms of BSE appear to be sporadic and thus may never be eradicated. A major challenge for prion surveillance is the lack of sufficiently practical and sensitive tests for routine BSE detection and strain discrimination. The real-time quaking-induced conversion (RT-QuIC) test, which is based on prion-seeded fibrillization of recombinant prion protein (rPrP Sen ), is known to be highly specific and sensitive for the detection of multiple human and animal prion diseases but not BSE. Here, we tested brain tissue from cattle affected by C-BSE and atypical L-type bovine spongiform encephalopathy (L-type BSE or L-BSE) with the RT-QuIC assay and found that both BSE forms can be detected and distinguished using particular rPrP Sen substrates. Specifically, L-BSE was detected using multiple rPrP Sen substrates, while C-BSE was much more selective. This substrate-based approach suggests a diagnostic strategy for specific, sensitive, and rapid detection and discrimination of at least some BSE forms.T ransmissible spongiform encephalopathies (TSEs), or prion diseases, such as Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE) in cattle, are fatal neurodegenerative disorders characterized by spongiosis, neuronal loss, gliosis, and abnormal deposition of host-encoded prion protein (PrP) in the brain. The infectious agent responsible for TSEs appears to be largely composed of a misfolded and multimeric form of prion protein (PrP Sc ), which is able to induce polymerization and conformational conversion of normal proteasesensitive prion protein (PrP Sen ) into PrP Sc and its partially protease-resistant forms (PrP Res ) (1). BSE and its link to variant Creutzfeldt-Jakob disease (vCJD) in humans have raised important food safety issues. The incidence of classical BSE (C-BSE) has decreased as a result of disease-control programs, such as the ruminant feed ban (2). However, atypical forms of bovine prion disease have been identified in several countries (3). These emerging bovine prion strains can be categorized as H-type BSE (H-BSE) or L-type BSE (L-BSE or bovine amyloidotic spongiform encephalopathy [BASE] when amyloid plaques are present in the brain). These atypical BSE strains are differentiated biochemically by the electrophoretic mobility and glycoform pattern of PrP Res after proteinase K (PK) digestion (4-7). Currently, both of these atypical bovine prion strains, which mainly affect older animals (8), are thought to be sporadic forms of bovine prion diseases (9) and are still rare (less than 100 cases identified worldwide to date).It is suspected that one of these atypical L-BSE or H-BSE strains may have instigated the C-BSE epidemic, as suggested, for example, by the fact that the L-BSE strain can convert to C-BSE following serial passage in mice (5,6,10). Further concerns about the atypical BSE strains have arisen from...

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“…M137T and N176K variants on the ARQ haplotype have been associated with scrapie resistance in intercranially-inoculated, orally-inoculated, and naturally-infected Italian Sarda breed sheep (Vaccari et al 2007;Vaccari et al 2009). Variations at codon 141 can be related to the atypical scrapie form Nor98 (Mazza et al 2010).…”

Section: Introductionmentioning

confidence: 99%

Single nucleotide polymorphisms at 15 codons of the prion protein gene from a scrapie-affected herd of Suffolk sheep in Brazil

et al. 2011

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Scrapie is a transmissible spongiform encephalopathy of sheeps and goats, associated with the deposition of a isoform of the prion protein (PrPsc). This isoform presents an altered conformation that leads to aggregation in the host's central nervous and lymphoreticular systems. Predisposition to the prion agent infection can be influenced by specific genotypes related to mutations in amino acids of the PrPsc gene. The most characterized mutations occur at codons 136, 154 and 171, with genotypes VRQ being the most susceptible and ARR the most resistant. In this study we have analyzed polymorphisms in 15 different codons of the PrPsc gene in sheeps from a Suffolk herd from Brazil affected by an outbreak of classical scrapie. Amplicons from the PrPsc gene, encompassing the most relevant altered codons in the protein, were sequenced in order to determine each animal's genotype. We have found polymorphisms at 3 of the 15 analyzed codons (136, 143 and 171). The most variable codon was 171, where all described alleles were identified. A rare polymorphism was found at the 143 codon in 4% of the samples analyzed, which has been described as increasing scrapie resistance in otherwise susceptible animals. No other polymorphisms were detected in the remaining 12 analyzed codons, all of them corresponding to the wild-type prion protein. Regarding the risk degree of developing scrapie, most of the animals (96%) had genotypes corresponding to risk groups 1 to 3 (very low to moderate), with only 4% in the higher risks group. Our data is discussed in relation to preventive measures involving genotyping and positive selection to control the disease.

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Co-existence of classical scrapie and Nor98 in a sheep from an Italian outbreak

Cited by 37 publications

References 48 publications

Archival search for historical atypical scrapie in sheep reveals evidence for mixed infections

Archival search for historical atypical scrapie in sheep reveals evidence for mixed infections

Detection and Discrimination of Classical and Atypical L-Type Bovine Spongiform Encephalopathy by Real-Time Quaking-Induced Conversion

Single nucleotide polymorphisms at 15 codons of the prion protein gene from a scrapie-affected herd of Suffolk sheep in Brazil

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