BSE Case Associated with Prion Protein Gene Mutation
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{"title"=>"BSE case associated with prion protein gene mutation", "type"=>"journal", "authors"=>[{"first_name"=>"Jürgen A.", "last_name"=>"Richt", "scopus_author_id"=>"7003289999"}, {"first_name"=>"S. Mark", "last_name"=>"Hall", "scopus_author_id"=>"12796536500"}], "year"=>2008, "source"=>"PLoS Pathogens", "identifiers"=>{"issn"=>"15537366", "pmid"=>"18787697", "scopus"=>"2-s2.0-53049106751", "sgr"=>"53049106751", "doi"=>"10.1371/journal.ppat.1000156", "pui"=>"352447257", "isbn"=>"1553-7374 (Electronic)"}, "id"=>"4991881a-138a-3075-8d76-cda70c8d3b59", "abstract"=>"Bovine spongiform encephalopathy (BSE) is a transmissible spongiform encephalopathy (TSE) of cattle and was first detected in 1986 in the United Kingdom. It is the most likely cause of variant Creutzfeldt-Jakob disease (CJD) in humans. The origin of BSE remains an enigma. Here we report an H-type BSE case associated with the novel mutation E211K within the prion protein gene (Prnp). Sequence analysis revealed that the animal with H-type BSE was heterozygous at Prnp nucleotides 631 through 633. An identical pathogenic mutation at the homologous codon position (E200K) in the human Prnp has been described as the most common cause of genetic CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. A recent epidemiological study revealed that the K211 allele was not detected in 6062 cattle from commercial beef processing plants and 42 cattle breeds, indicating an extremely low prevalence of the E211K variant (less than 1 in 2000) in cattle.", "link"=>"http://www.mendeley.com/research/bse-case-associated-prion-protein-gene-mutation-6", "reader_count"=>20, "reader_count_by_academic_status"=>{"Professor > Associate Professor"=>1, "Researcher"=>4, "Student > Doctoral Student"=>3, "Student > Ph. D. Student"=>2, "Student > Postgraduate"=>1, "Student > Master"=>3, "Other"=>1, "Student > Bachelor"=>5}, "reader_count_by_user_role"=>{"Professor > Associate Professor"=>1, "Researcher"=>4, "Student > Doctoral Student"=>3, "Student > Ph. D. Student"=>2, "Student > Postgraduate"=>1, "Student > Master"=>3, "Other"=>1, "Student > Bachelor"=>5}, "reader_count_by_subject_area"=>{"Unspecified"=>1, "Biochemistry, Genetics and Molecular Biology"=>1, "Nursing and Health Professions"=>1, "Medicine and Dentistry"=>2, "Agricultural and Biological Sciences"=>13, "Veterinary Science and Veterinary Medicine"=>2}, "reader_count_by_subdiscipline"=>{"Medicine and Dentistry"=>{"Medicine and Dentistry"=>2}, "Agricultural and Biological Sciences"=>{"Agricultural and Biological Sciences"=>13}, "Nursing and Health Professions"=>{"Nursing and Health Professions"=>1}, "Biochemistry, Genetics and Molecular Biology"=>{"Biochemistry, Genetics and Molecular Biology"=>1}, "Unspecified"=>{"Unspecified"=>1}, "Veterinary Science and Veterinary Medicine"=>{"Veterinary Science and Veterinary Medicine"=>2}}, "group_count"=>0}

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Figshare

  • {"files"=>["https://ndownloader.figshare.com/files/921912"], "description"=>"<p>(A) Hybrid Immunoblot Analysis using enriched samples: Lanes 1–5: monoclonal antibody 6H4 (raised against human PrP residues 144–152), lanes 8–12 monoclonal antibody P4 (raised against ovine PrP residues 89–104): 1 = sheep scrapie control, 2 mg; 2 = classical BSE (2003 U.S. BSE case), 2 mg; 3 = H-type BSE case (2004 U.S. BSE case), 2 mg; 4 = U.S. BSE Alabama case, 1 mg; 5 = U.S. BSE Alabama case, 2.5 mg; 6,7 = protein weight maker; 8 = U.S. BSE Alabama case, 2.5 mg; 9 = U.S. BSE Alabama case, 1 mg; 10 = H-type BSE case (2004 U.S. BSE case), 2 mg; 11 = classical BSE (2003 U.S. BSE case), 2 mg; 12 = sheep scrapie control, 2 mg. (B) Immunohistochemistry of the U.S. BSE Alabama case (H-type BSE) using PrP-specific monoclonal antibody F99/97.6.1. Brainstem at the level of obex was examined. Bar = 35 µm. (C) Immunohistochemistry of a classical BSE case <a href=\"http://www.plospathogens.org/article/info:doi/10.1371/journal.ppat.1000156#ppat.1000156-Richt1\" target=\"_blank\">[4]</a> using PrP-specific monoclonal antibody F99/97.6.1. Brainstem at the level of obex was examined. Spongiform changes are found in the area with highly PrP<sup>d</sup>-positive cells. Bar = 90 µm.</p>", "links"=>[], "tags"=>["brainstem", "samples", "bse-infected", "animals", "employing"], "article_id"=>592349, "categories"=>["Computational Biology", "Genetics", "Evolutionary Biology", "Microbiology", "Biochemistry", "Medicine", "Neuroscience"], "users"=>["Jürgen A. Richt", "S. Mark Hall"], "doi"=>"https://dx.doi.org/10.1371/journal.ppat.1000156.g001", "stats"=>{"downloads"=>1, "page_views"=>12, "likes"=>0}, "figshare_url"=>"https://figshare.com/articles/_Analysis_of_brainstem_samples_from_BSE_infected_animals_employing_various_methods_/592349", "title"=>"Analysis of brainstem samples from BSE-infected animals employing various methods.", "pos_in_sequence"=>0, "defined_type"=>1, "published_date"=>"2013-02-21 09:32:42"}
  • {"files"=>["https://ndownloader.figshare.com/files/922232"], "description"=>"<p>DNA sequence analysis of codon 211 of the bovine <i>Prnp</i> and codon 200 of the human <i>Prnp</i>.</p>", "links"=>[], "tags"=>["codon", "211", "bovine", "200"], "article_id"=>592681, "categories"=>["Computational Biology", "Genetics", "Evolutionary Biology", "Microbiology", "Biochemistry", "Medicine", "Neuroscience"], "users"=>["Jürgen A. Richt", "S. Mark Hall"], "doi"=>"https://dx.doi.org/10.1371/journal.ppat.1000156.t001", "stats"=>{"downloads"=>1, "page_views"=>7, "likes"=>0}, "figshare_url"=>"https://figshare.com/articles/_DNA_sequence_analysis_of_codon_211_of_the_bovine_Prnp_and_codon_200_of_the_human_Prnp_/592681", "title"=>"DNA sequence analysis of codon 211 of the bovine <i>Prnp</i> and codon 200 of the human <i>Prnp</i>.", "pos_in_sequence"=>0, "defined_type"=>3, "published_date"=>"2013-02-21 09:34:37"}
  • {"files"=>["https://ndownloader.figshare.com/files/922136"], "description"=>"<p>(A) Nucleotide sequences. Standard single letter codes are used for nucleotides. Y = C or T; R = A or G; K = G or T; W = A or T. Boxed area indicates the 6<sup>th</sup> octapeptide-repeat of the bovine protein (U.S. BSE Alabama case and sequence AY335912). Additional <i>Prnp</i> sequences are as follows: AJ567986 (sheep), AF016227 (elk), Hsap M13899 (human, normal) and Hsap PRNPvar [human, variant; see <a href=\"http://www.plospathogens.org/article/info:doi/10.1371/journal.ppat.1000156#ppat.1000156-Coulthart1\" target=\"_blank\">[13]</a>]. (B) Amino acid sequences. Standard IUPAC single letter codes are used for amino acids. Codon numbering refers to the most common six-copy octapeptide repeat allele for <i>Bos Taurus</i>. Boxed area indicates the 6<sup>th</sup> octapeptide repeat of the bovine protein [animals B14842 <a href=\"http://www.plospathogens.org/article/info:doi/10.1371/journal.ppat.1000156#ppat.1000156-Richt1\" target=\"_blank\">[4]</a> and AY335912]. AJ567986 (sheep), AF016227 (elk), Hsap M13899 (human, normal) and Hsap PRNPvar [human, variant; see <a href=\"http://www.plospathogens.org/article/info:doi/10.1371/journal.ppat.1000156#ppat.1000156-Coulthart1\" target=\"_blank\">[13]</a>] each contain a 5 octapeptide repeat region in the protein.</p>", "links"=>[], "tags"=>["cervid"], "article_id"=>592589, "categories"=>["Computational Biology", "Genetics", "Evolutionary Biology", "Microbiology", "Biochemistry", "Medicine", "Neuroscience"], "users"=>["Jürgen A. Richt", "S. Mark Hall"], "doi"=>"https://dx.doi.org/10.1371/journal.ppat.1000156.g002", "stats"=>{"downloads"=>1, "page_views"=>10, "likes"=>0}, "figshare_url"=>"https://figshare.com/articles/_Alignment_of_bovine_ovine_cervid_and_human_Prnp_sequences_/592589", "title"=>"Alignment of bovine, ovine, cervid and human <i>Prnp</i> sequences.", "pos_in_sequence"=>0, "defined_type"=>1, "published_date"=>"2013-02-21 09:34:10"}

PMC Usage Stats | Further Information

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Relative Metric

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