{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T18:00:56Z","timestamp":1777658456688,"version":"3.51.4"},"reference-count":19,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,11,3]],"date-time":"2023-11-03T00:00:00Z","timestamp":1698969600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Vet. Sci."],"abstract":"<jats:p>In 2006, a case of atypical H-type BSE (H-BSE) was found to be associated with a germline mutation in the <jats:italic>PRNP<\/jats:italic> gene that resulted in a lysine substitution for glutamic acid at codon 211 (E211K). The E211K amino acid substitution in cattle is analogous to E200K in humans, which is associated with the development of genetic Creutzfeldt-Jakob disease (CJD). In the present study, we aimed to determine the effect of the EK211 prion protein genotype on incubation time in cattle inoculated with the agent of H-BSE; to characterize the molecular profile of H-BSE in KK211 and EK211 genotype cattle; and to assess the influence of serial passage on BSE strain. Eight cattle, representing three <jats:italic>PRNP<\/jats:italic> genotype groups (EE211, EK211, and KK211), were intracranially inoculated with the agent of H-BSE originating from either a case in a cow with the EE211 prion protein genotype or a case in a cow with E211K amino acid substitution. All inoculated animals developed clinical disease; post-mortem samples were collected, and prion disease was confirmed through enzyme immunoassay, anti-PrP<jats:sup>Sc<\/jats:sup> immunohistochemistry, and western blot. Western blot molecular analysis revealed distinct patterns in a steer with KK211 H-BSE compared to EK211 and EE211 cattle. Incubation periods were significantly shorter in cattle with the EK211 and KK211 genotypes compared to the EE211 genotype. Inoculum type did not significantly influence the incubation period. This study demonstrates a shorter incubation period for H-BSE in cattle with the K211 genotype in both the homozygous and heterozygous forms.<\/jats:p>","DOI":"10.3389\/fvets.2023.1301998","type":"journal-article","created":{"date-parts":[[2023,11,3]],"date-time":"2023-11-03T09:15:22Z","timestamp":1699002922000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":4,"title":["Short incubation periods of atypical H-type BSE in cattle with EK211 and KK211 prion protein genotypes after intracranial inoculation"],"prefix":"10.3389","volume":"10","author":[{"given":"Eric D.","family":"Cassmann","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexis J.","family":"Frese","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kelsey A.","family":"Becker","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Justin J.","family":"Greenlee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2023,11,3]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"110","DOI":"10.1038\/sj.embor.7400054","article-title":"Distinct molecular phenotypes in bovine prion diseases","volume":"5","author":"Biacabe","year":"2004","journal-title":"EMBO Rep."},{"key":"B2","doi-asserted-by":"publisher","first-page":"823","DOI":"10.1177\/104063871002200601","article-title":"Atypical transmissible spongiform encephalopathies in ruminants: a challenge for disease surveillance and control","volume":"22","author":"Seuberlich","year":"2010","journal-title":"J Vet Diagn Invest."},{"key":"B3","doi-asserted-by":"publisher","first-page":"61","DOI":"10.4161\/pri.1.1.3828","article-title":"H-type bovine spongiform encephalopathy: complex molecular features and similarities with human prion diseases","volume":"1","author":"Biacabe","year":"2007","journal-title":"Prion."},{"key":"B4","doi-asserted-by":"publisher","first-page":"1816","DOI":"10.3201\/eid1212.060965","article-title":"On the question of sporadic or atypical bovine spongiform encephalopathy and Creutzfeldt-Jakob disease","volume":"12","author":"Brown","year":"2006","journal-title":"Emerg Infect Dis."},{"key":"B5","doi-asserted-by":"publisher","first-page":"e1000156","DOI":"10.1371\/journal.ppat.1000156","article-title":"BSE case associated with prion protein gene mutation","volume":"4","author":"Richt","year":"2008","journal-title":"PLoS Pathog."},{"key":"B6","doi-asserted-by":"publisher","first-page":"e1830","DOI":"10.1371\/journal.pone.0001830","article-title":"Association of a bovine prion gene haplotype with atypical BSE","volume":"3","author":"Clawson","year":"2008","journal-title":"PLoS ONE."},{"key":"B7","doi-asserted-by":"publisher","first-page":"e2912","DOI":"10.1371\/journal.pone.0002912","article-title":"Identification of a heritable polymorphism in bovine PRNP associated with genetic transmissible spongiform encephalopathy: evidence of heritable BSE","volume":"3","author":"Nicholson","year":"2008","journal-title":"PloS ONE."},{"key":"B8","doi-asserted-by":"publisher","first-page":"1","DOI":"10.3389\/fvets.2016.00078","article-title":"Comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild-type and EK211 cattle following intracranial inoculation","volume":"3","author":"Moore","year":"2016","journal-title":"Front Vet Sci."},{"key":"B9","doi-asserted-by":"publisher","first-page":"1636","DOI":"10.3201\/eid1709.101403","article-title":"Classical bovine spongiform encephalopathy by transmission of H-type prion in homologous prion protein context","volume":"17","author":"Torres","year":"2011","journal-title":"Emerg Infect Dis."},{"key":"B10","doi-asserted-by":"publisher","first-page":"e15839","DOI":"10.1371\/journal.pone.0015839","article-title":"Emergence of classical BSE strain properties during serial passages of H-BSE in wild-type mice","volume":"6","author":"Baron","year":"2011","journal-title":"PLoS ONE."},{"key":"B11","doi-asserted-by":"publisher","first-page":"94","DOI":"10.3389\/fvets.2020.00094","article-title":"Influence of interspecies transmission of atypical bovine spongiform encephalopathy prions to hamsters on prion characteristics","volume":"7","author":"Miyazawa","year":"2020","journal-title":"Front Vet Sci."},{"key":"B12","doi-asserted-by":"publisher","first-page":"142","DOI":"10.1177\/104063870701900202","article-title":"Identification and characterization of two bovine spongiform encephalopathy cases diagnosed in the United States","volume":"19","author":"Richt","year":"2007","journal-title":"J Vet Diagn Invest."},{"key":"B13","doi-asserted-by":"publisher","first-page":"e38678","DOI":"10.1371\/journal.pone.0038678","article-title":"Clinical and pathologic features of H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism","volume":"7","author":"Greenlee","year":"2012","journal-title":"PLoS ONE."},{"key":"B14","doi-asserted-by":"publisher","first-page":"10406387211017615","DOI":"10.1177\/10406387211017615","article-title":"Second passage of chronic wasting disease of mule deer to sheep by intracranial inoculation compared to classical scrapie","volume":"12","author":"Cassmann","year":"2021","journal-title":"J Vet Diagn Invest"},{"key":"B15","doi-asserted-by":"publisher","first-page":"e0119431","DOI":"10.1371\/journal.pone.0119431","article-title":"Changes in retinal function and morphology are early clinical signs of disease in cattle with bovine spongiform encephalopathy","volume":"10","author":"West Greenlee","year":"2015","journal-title":"PLoS one."},{"key":"B16","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1186\/1297-9716-43-55","article-title":"Susceptibility to scrapie and disease phenotype in sheep: cross-PRNP genotype experimental transmissions with natural sources","volume":"43","author":"Gonzalez","year":"2012","journal-title":"Vet Res."},{"key":"B17","doi-asserted-by":"publisher","first-page":"1003056","DOI":"10.3389\/fneur.2022.1003056","article-title":"Genetic aspects of human prion diseases","volume":"13","author":"Appleby","year":"2022","journal-title":"Front Neurol."},{"key":"B18","doi-asserted-by":"publisher","first-page":"12","DOI":"10.3390\/ijms21124246","article-title":"First report of the potential bovine spongiform encephalopathy (BSE)-related somatic mutation E211K of the prion protein gene (PRNP) in cattle","volume":"21","author":"Won","year":"2020","journal-title":"Int J Mol Sci."},{"key":"B19","doi-asserted-by":"publisher","first-page":"1821","DOI":"10.1128\/JCM.00160-07","article-title":"Molecular discrimination of atypical bovine spongiform encephalopathy strains from a geographical region spanning a wide area in Europe","volume":"45","author":"Jacobs","year":"2007","journal-title":"J Clin Microbiol."}],"container-title":["Frontiers in Veterinary Science"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fvets.2023.1301998\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,3]],"date-time":"2023-11-03T09:15:25Z","timestamp":1699002925000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fvets.2023.1301998\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,11,3]]},"references-count":19,"alternative-id":["10.3389\/fvets.2023.1301998"],"URL":"https:\/\/doi.org\/10.3389\/fvets.2023.1301998","relation":{},"ISSN":["2297-1769"],"issn-type":[{"value":"2297-1769","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,11,3]]},"article-number":"1301998"}}