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Douet, Jean-Yves; Huor, Alvina; Cassard, Hervé; Lugan, Séverine; Aron, Naïma; Mesic, Chloé; Vilette, Didier; Barrio, Tomás; Streichenberger, Nathalie; Perret-Liaudet, Armand; Delisle, Marie-Bernadette; Péran, Patrice; Deslys, Jean-Philippe; Comoy, Emmanuel; Vilotte, Jean-Luc; Goudarzi, Katayoun; Béringue, Vincent; Barria, Marcelo A.; Ritchie, Diane L.; Ironside, James W.; Andréoletti, Olivier

Prion strains associated with iatrogenic CJD in French and UK human growth hormone recipients

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  • Media type: E-Article
  • Title: Prion strains associated with iatrogenic CJD in French and UK human growth hormone recipients
  • Contributor: Douet, Jean-Yves; Huor, Alvina; Cassard, Hervé; Lugan, Séverine; Aron, Naïma; Mesic, Chloé; Vilette, Didier; Barrio, Tomás; Streichenberger, Nathalie; Perret-Liaudet, Armand; Delisle, Marie-Bernadette; Péran, Patrice; Deslys, Jean-Philippe; Comoy, Emmanuel; Vilotte, Jean-Luc; Goudarzi, Katayoun; Béringue, Vincent; Barria, Marcelo A.; Ritchie, Diane L.; Ironside, James W.; Andréoletti, Olivier
  • imprint: Springer Science and Business Media LLC, 2021
  • Published in: Acta Neuropathologica Communications
  • Language: English
  • DOI: 10.1186/s40478-021-01247-x
  • ISSN: 2051-5960
  • Keywords: Cellular and Molecular Neuroscience ; Neurology (clinical) ; Pathology and Forensic Medicine
  • Origination:
  • Footnote:
  • Description: <jats:title>Abstract</jats:title><jats:p>Treatment with human pituitary-derived growth hormone (hGH) was responsible for a significant proportion of iatrogenic Creutzfeldt–Jakob disease (iCJD) cases. France and the UK experienced the largest case numbers of hGH-iCJD, with 122 and 81 cases respectively. Differences in the frequency of the three <jats:italic>PRNP</jats:italic> codon 129 polymorphisms (MM, MV and VV) and the estimated incubation periods associated with each of these genotypes in the French and the UK hGH-iCJD cohorts led to the suggestion that the prion strains responsible for these two hGH-iCJD cohorts were different. In this study, we characterized the prion strains responsible for hGH-iCJD cases originating from UK (n = 11) and France (n = 11) using human PrP expressing mouse models. The cases included <jats:italic>PRNP</jats:italic> MM, MV and VV genotypes from both countries. UK and French sporadic CJD (sCJD) cases were included as controls. The prion strains identified following inoculation with hGH-iCJD homogenates corresponded to the two most frequently observed sCJD prion strains (M1<jats:sup>CJD</jats:sup> and V2<jats:sup>CJD</jats:sup>). However, in clear contradiction to the initial hypothesis, the prion strains that were identified in the UK and the French hGH-iCJD cases were not radically different. In the vast majority of the cases originating from both countries, the V2<jats:sup>CJD</jats:sup> strain or a mixture of M1<jats:sup>CJD</jats:sup> + V2<jats:sup>CJD</jats:sup> strains were identified. These data strongly support the contention that the differences in the epidemiological and genetic profiles observed in the UK and France hGH-iCJD cohorts cannot be attributed only to the transmission of different prion strains.</jats:p>
  • Access State: Open Access