Literature DB >> 19324055

Scrapie-infected transgenic mice expressing a laminin receptor decoy mutant reveal a prolonged incubation time associated with low levels of PrPres.

Heike Pflanz1, Karen Vana, Gerda Mitteregger, Ingrid Renner-Müller, Claudia Pace, Helmut Küchenhoff, Hans A Kretzschmar, Eckhard Wolf, Stefan Weiss.   

Abstract

The 37-kDa/67-kDa laminin receptor (LRP/LR) was identified as a cell surface receptor for prion proteins. The laminin receptor mutant LRP102-295::FLAG interfered with PrP(Sc) propagation in murine neuronal cells presumably acting as a decoy in a transdominant negative fashion by trapping PrP molecules in the extracellular matrix. Here, we generated hemizygous transgenic mice expressing LRP102-295::FLAG in the brain. Scrapie-infected transgenic mice exhibit a significantly prolonged incubation time in comparison to scrapie-infected wild-type (FVB) mice. At the terminal stage, transgenic mice revealed significantly reduced proteinase-K-resistant PrP levels by 71% compared to wild-type mice. Our results recommend the laminin receptor decoy mutant as an alternative therapeutic tool for treatment of transmissible spongiform encephalopathies.

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Year:  2009        PMID： 19324055     DOI： 10.1016/j.jmb.2009.03.045

Source DB:  PubMed          Journal:  J Mol Biol        ISSN： 0022-2836            Impact factor:   5.469


  7 in total

1.  Neuronal low-density lipoprotein receptor-related protein 1 binds and endocytoses prion fibrils via receptor cluster 4.

Authors:  Angela Jen; Celia J Parkyn; Roy C Mootoosamy; Melanie J Ford; Alice Warley; Qiang Liu; Guojun Bu; Ilia V Baskakov; Søren Moestrup; Lindsay McGuinness; Nigel Emptage; Roger J Morris
Journal:  J Cell Sci       Date:  2010-01-15       Impact factor: 5.285

2.  MEK1 transduces the prion protein N2 fragment antioxidant effects.

Authors:  C L Haigh; A R McGlade; S J Collins
Journal:  Cell Mol Life Sci       Date:  2014-11-13       Impact factor: 9.261

3.  Characterization of the ovine ribosomal protein SA gene and its pseudogenes.

Authors:  Alice Van den Broeke; Mario Van Poucke; Ane Marcos-Carcavilla; Karine Hugot; Hélène Hayes; Maud Bertaud; Alex Van Zeveren; Luc J Peelman
Journal:  BMC Genomics       Date:  2010-03-16       Impact factor: 3.969

4.  Fatal transmissible amyloid encephalopathy: a new type of prion disease associated with lack of prion protein membrane anchoring.

Authors:  Bruce Chesebro; Brent Race; Kimberly Meade-White; Rachel Lacasse; Richard Race; Mikael Klingeborn; James Striebel; David Dorward; Gillian McGovern; Martin Jeffrey
Journal:  PLoS Pathog       Date:  2010-03-05       Impact factor: 6.823

5.  Expression and distribution of laminin receptor precursor/laminin receptor in rabbit tissues.

Authors:  Huinuan Wang; Lifeng Yang; Mohammed Kouadir; Rongrong Tan; Wenyu Wu; Huarong Zou; Jin Wang; Sher Hayat Khan; Dongfeng Li; Xiangmei Zhou; Xiaomin Yin; Yunsheng Wang; Deming Zhao
Journal:  J Mol Neurosci       Date:  2013-05-30       Impact factor: 3.444

6.  The first report of RPSA polymorphisms, also called 37/67 kDa LRP/LR gene, in sporadic Creutzfeldt-Jakob disease (CJD).

Authors:  Jisuk Yun; Hyoung-Tae Jin; Yun-Jung Lee; Eun-Kyoung Choi; Richard I Carp; Byung-Hoon Jeong; Yong-Sun Kim
Journal:  BMC Med Genet       Date:  2011-08-13       Impact factor: 2.103

7.  Interactome analyses identify ties of PrP and its mammalian paralogs to oligomannosidic N-glycans and endoplasmic reticulum-derived chaperones.

Authors:  Joel C Watts; Hairu Huo; Yu Bai; Sepehr Ehsani; Amy Hye Won Jeon; Amy Hye Won; Tujin Shi; Nathalie Daude; Agnes Lau; Rebecca Young; Lei Xu; George A Carlson; David Williams; David Westaway; Gerold Schmitt-Ulms
Journal:  PLoS Pathog       Date:  2009-10-02       Impact factor: 6.823
  7 in total

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