The Expanding Universe of Prion Diseases
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{"title"=>"The expanding universe of prion diseases", "type"=>"generic", "authors"=>[{"first_name"=>"Joel C.", "last_name"=>"Watts", "scopus_author_id"=>"8789165800"}, {"first_name"=>"Aru", "last_name"=>"Balachandran", "scopus_author_id"=>"35611360000"}, {"first_name"=>"David", "last_name"=>"Westaway", "scopus_author_id"=>"7006295116"}], "year"=>2006, "source"=>"PLoS Pathogens", "identifiers"=>{"scopus"=>"2-s2.0-33645796939", "pmid"=>"16609731", "sgr"=>"33645796939", "doi"=>"10.1371/journal.ppat.0020026", "isbn"=>"1553-7374", "issn"=>"15537366", "pui"=>"43555749"}, "id"=>"8893e7fc-6dc4-3474-bd1f-3b7966dd32ba", "abstract"=>"Prions cause fatal and transmissible neurodegenerative disease. These etiological infectious agents are formed in greater part from a misfolded cell-surface protein called PrP(C). Several mammalian species are affected by the diseases, and in the case of \"mad cow disease\" (BSE) the agent has a tropism for humans, with negative consequences for agribusiness and public health. Unfortunately, the known universe of prion diseases is expanding. At least four novel prion diseases--including human diseases variant Creutzfeldt-Jakob disease (vCJD) and sporadic fatal insomnia (sFI), bovine amyloidotic spongiform encephalopathy (BASE), and Nor98 of sheep--have been identified in the last ten years, and chronic wasting disease (CWD) of North American deer (Odocoileus Specis) and Rocky Mountain elk (Cervus elaphus nelsoni) is undergoing a dramatic spread across North America. While amplification (BSE) and dissemination (CWD, commercial sourcing of cervids from the wild and movement of farmed elk) can be attributed to human activity, the origins of emergent prion diseases cannot always be laid at the door of humankind. Instead, the continued appearance of new outbreaks in the form of \"sporadic\" disease may be an inevitable outcome in a situation where the replicating pathogen is host-encoded.", "link"=>"http://www.mendeley.com/research/expanding-universe-prion-diseases", "reader_count"=>78, "reader_count_by_academic_status"=>{"Unspecified"=>1, "Professor > Associate Professor"=>2, "Researcher"=>19, "Student > Doctoral Student"=>6, "Student > Ph. D. Student"=>12, "Student > Postgraduate"=>4, "Other"=>6, "Student > Master"=>6, "Student > Bachelor"=>13, "Lecturer"=>1, "Lecturer > Senior Lecturer"=>3, "Professor"=>5}, "reader_count_by_user_role"=>{"Unspecified"=>1, "Professor > Associate Professor"=>2, "Researcher"=>19, "Student > Doctoral Student"=>6, "Student > Ph. D. Student"=>12, "Student > Postgraduate"=>4, "Other"=>6, "Student > Master"=>6, "Student > Bachelor"=>13, "Lecturer"=>1, "Lecturer > Senior Lecturer"=>3, "Professor"=>5}, "reader_count_by_subject_area"=>{"Unspecified"=>2, "Environmental Science"=>2, "Biochemistry, Genetics and Molecular Biology"=>6, "Agricultural and Biological Sciences"=>47, "Medicine and Dentistry"=>14, "Neuroscience"=>1, "Veterinary Science and Veterinary Medicine"=>2, "Psychology"=>1, "Chemistry"=>1, "Immunology and Microbiology"=>2}, "reader_count_by_subdiscipline"=>{"Medicine and Dentistry"=>{"Medicine and Dentistry"=>14}, "Neuroscience"=>{"Neuroscience"=>1}, "Chemistry"=>{"Chemistry"=>1}, "Psychology"=>{"Psychology"=>1}, "Immunology and Microbiology"=>{"Immunology and Microbiology"=>2}, "Agricultural and Biological Sciences"=>{"Agricultural and Biological Sciences"=>47}, "Biochemistry, Genetics and Molecular Biology"=>{"Biochemistry, Genetics and Molecular Biology"=>6}, "Unspecified"=>{"Unspecified"=>2}, "Environmental Science"=>{"Environmental Science"=>2}, "Veterinary Science and Veterinary Medicine"=>{"Veterinary Science and Veterinary Medicine"=>2}}, "reader_count_by_country"=>{"Canada"=>1, "United States"=>1, "China"=>1, "Ireland"=>1, "Denmark"=>1, "United Kingdom"=>1, "Slovenia"=>1, "France"=>1, "Portugal"=>1}, "group_count"=>3}

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Figshare

  • {"files"=>["https://ndownloader.figshare.com/files/959625"], "description"=>"<p>Brain homogenate from an infected animal (containing PrP<sup>Sc</sup>) is diluted into homogenate from a healthy animal (containing PrP<sup>C</sup>) and incubated at 37 °C. During this stage, some molecules of PrP<sup>C</sup> are converted to PrP<sup>res</sup> and are added to the growing PrP<sup>res</sup> unit. Sonication breaks up PrP<sup>res</sup> into smaller units, generating new seeds for conversion. These steps are repeated in a cyclic fashion in order to amplify the amount of PrP<sup>res</sup> present in the initial sample. The stoichiometry and directionality of amplification depicted are for illustrative purposes only and are not meant to represent intrinsic properties of the system.</p>", "links"=>[], "tags"=>["pmca"], "article_id"=>629651, "categories"=>["Biochemistry", "Neuroscience", "Cell Biology", "Infectious Diseases"], "users"=>["Joel C Watts", "Aru Balachandran", "David Westaway"], "doi"=>"https://dx.doi.org/10.1371/journal.ppat.0020026.g003", "stats"=>{"downloads"=>1, "page_views"=>7, "likes"=>0}, "figshare_url"=>"https://figshare.com/articles/_Schematic_Representation_of_the_PMCA_Procedure_/629651", "title"=>"Schematic Representation of the PMCA Procedure", "pos_in_sequence"=>0, "defined_type"=>1, "published_date"=>"2013-02-22 08:44:06"}
  • {"files"=>["https://ndownloader.figshare.com/files/959395"], "description"=>"<div><p>(A–C) Structures of PrP<sup>C</sup> and PrP<sup>Sc</sup>.</p><p>(A) NMR structure of PrP<sup>C</sup> with a notional addition of the N-terminal region (which is unstructured in NMR analyses). Copper ions (turquoise spheres) and coordinating side chains (dark blue) are shown. Note that the C-terminal globular domain has two short beta strands (turquoise arrows) and three α-helices (red; image courtesy of Glenn Millhauser, University of California Santa Cruz).</p><p>(B) A structure for PrP<sup>Sc</sup> deduced from electron microscopic analyses of purified material. The background represents the two-dimensional crystals of PrP27–30 after image processing and the foreground a notional structure for PrP<sup>Sc</sup> with the central region of the protein rearranged into triangular stacked β-helices (image courtesy of Cedric Govaerts and Holger Wille, University of California San Francisco).</p><p>(C) Linear representation of PrP<sup>C</sup> and PrP<sup>Sc</sup>, and N-terminal truncation of PrP<sup>Sc</sup> by proteinase K, to create PrP27–30. For illustrative purposes the conformational change in the centre of the molecule is shown by an altered color for helix A. Also note that other conformational changes may occur C-terminal to this position, but have been omitted for the sake of clarity.</p><p>(D) Pathways to disease. In prion infections, conversion of PrP<sup>C</sup> to PrP<sup>Sc</sup> is initiated by a pre-existing seed of PrP<sup>Sc</sup>. In genetic diseases the mutant form of PrP<sup>C</sup> denoted PrP<sup>M</sup> may misfold and aggregate, and in some case acquire PrP<sup>Sc</sup>-like properties sufficient to initiate disease in inoculated recipient animals. In sporadic disease the mechanism of spontaneous misfolding to PrP<sup>Sc</sup> is unknown.</p></div>", "links"=>[], "tags"=>["pathways"], "article_id"=>629425, "categories"=>["Biochemistry", "Neuroscience", "Cell Biology", "Infectious Diseases"], "users"=>["Joel C Watts", "Aru Balachandran", "David Westaway"], "doi"=>"https://dx.doi.org/10.1371/journal.ppat.0020026.g001", "stats"=>{"downloads"=>0, "page_views"=>0, "likes"=>0}, "figshare_url"=>"https://figshare.com/articles/_Formation_of_PrP_Sc_and_Pathways_to_Disease_/629425", "title"=>"Formation of PrP<sup>Sc</sup> and Pathways to Disease", "pos_in_sequence"=>0, "defined_type"=>1, "published_date"=>"2013-02-22 08:42:26"}
  • {"files"=>["https://ndownloader.figshare.com/files/959500"], "description"=>"<div><p>(A) Histologic lesions of CWD in the dorsal motor nuclei of the vagus nerve within the medulla oblongata of a CWD-affected elk. Note spongiform change, intraneuronal vacuoles, and mild gliosis (hematoxylin and eosin stain, 100× magnification).</p><p>(B) PrP amyloid deposit stained by immunohistochemistry (brown) and surrounded by vacuoles (original magnification 180×).</p><p>(C)<b> </b> Perineuronal and extracellular deposits of abnormal PrP (PrP<sup>CWD</sup>, analogous to PrP<sup>Sc</sup>) in a CWD-affected mule deer (original magnification180×).</p><p>(D) PrP<sup>CWD</sup> deposits in the germinal centres of lymphoid follicles in the tonsils of a mule deer (immunohistochemistry, original magnification 50×).</p></div>", "links"=>[], "tags"=>["cwd-infected"], "article_id"=>629530, "categories"=>["Biochemistry", "Neuroscience", "Cell Biology", "Infectious Diseases"], "users"=>["Joel C Watts", "Aru Balachandran", "David Westaway"], "doi"=>"https://dx.doi.org/10.1371/journal.ppat.0020026.g002", "stats"=>{"downloads"=>0, "page_views"=>6, "likes"=>0}, "figshare_url"=>"https://figshare.com/articles/_Pathology_in_CWD_Infected_Animals_/629530", "title"=>"Pathology in CWD-Infected Animals", "pos_in_sequence"=>0, "defined_type"=>1, "published_date"=>"2013-02-22 08:43:14"}
  • {"files"=>["https://ndownloader.figshare.com/files/959756"], "description"=>"<div><p>Geographical distribution in North America (A), and in expanded view of Alberta and Saskatchewan (B). Data in (A) is correct as of February, 2006, and reproduced with kind permission of the CWD Alliance.</p><p>In (A), states or provinces where CWD has been diagnosed in captive populations are cross-hatched. CWD diagnosis in free-ranging animals is indicated by red circles.</p><p>In (B), CWD diagnosis in free-ranging animals is indicated by red circles and in farmed animals with green circles.</p><p>(C) Mule deer with clinical CWD showing emaciation and loss of body condition (photo courtesy of T. R. Spraker).</p><p>(D) Elk with clinical CWD showing emaciation and excessive salivation (photo courtesy of E. S. Williams).</p></div>", "links"=>[], "tags"=>["Biochemistry", "cell biology", "Infectious diseases", "neuroscience"], "article_id"=>629775, "categories"=>["Biochemistry", "Neuroscience", "Cell Biology", "Infectious Diseases"], "users"=>["Joel C Watts", "Aru Balachandran", "David Westaway"], "doi"=>"https://dx.doi.org/10.1371/journal.ppat.0020026.g004", "stats"=>{"downloads"=>1, "page_views"=>6, "likes"=>0}, "figshare_url"=>"https://figshare.com/articles/_CWD_in_North_America_/629775", "title"=>"CWD in North America", "pos_in_sequence"=>0, "defined_type"=>1, "published_date"=>"2013-02-22 08:45:04"}

PMC Usage Stats | Further Information

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Relative Metric

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