Selective neuronal targeting in prion disease - PubMed (original) (raw)

. 1997 Dec;19(6):1337-48.

doi: 10.1016/s0896-6273(00)80424-9.

H Sánchez, F Yehiely, Y Qiu, A Ninchak-Casey, V Daggett, A P Camerino, J Cayetano, M Rogers, D Groth, M Torchia, P Tremblay, M R Scott, F E Cohen, S B Prusiner

Affiliations

• PMID: 9427256
• DOI: 10.1016/s0896-6273(00)80424-9

Free article

Selective neuronal targeting in prion disease

S J DeArmond et al. Neuron. 1997 Dec.

Free article

Abstract

The pattern of scrapie prion protein (PrP(Sc)) accumulation in the brain is different for each prion strain. We tested whether the PrP(Sc) deposition pattern is influenced by the Asn-linked oligosaccharides of PrP(C) in transgenic mice. Deletion of the first oligosaccharide altered PrP(C) trafficking and prevented infection with two prion strains. Deletion of the second did not alter PrP(C) trafficking, permitted infection with one prion strain, and had a profound effect on the PrP(Sc) deposition pattern. Our data raise the possibility that glycosylation can modify the conformation of PrP(C). Glycosylation could affect the affinity of PrP(C) for a particular conformer of PrP(Sc), thereby determining the rate of nascent PrP(Sc) formation and the specific patterns of PrP(Sc) deposition.

PubMed Disclaimer

Similar articles

• Prion Protein Devoid of the Octapeptide Repeat Region Delays Bovine Spongiform Encephalopathy Pathogenesis in Mice.
  Hara H, Miyata H, Das NR, Chida J, Yoshimochi T, Uchiyama K, Watanabe H, Kondoh G, Yokoyama T, Sakaguchi S. Hara H, et al. J Virol. 2017 Dec 14;92(1):e01368-17. doi: 10.1128/JVI.01368-17. Print 2018 Jan 1. J Virol. 2017. PMID: 29046443 Free PMC article.
• PrPc glycoform heterogeneity as a function of brain region: implications for selective targeting of neurons by prion strains.
  DeArmond SJ, Qiu Y, Sànchez H, Spilman PR, Ninchak-Casey A, Alonso D, Daggett V. DeArmond SJ, et al. J Neuropathol Exp Neurol. 1999 Sep;58(9):1000-9. doi: 10.1097/00005072-199909000-00010. J Neuropathol Exp Neurol. 1999. PMID: 10499442
• The N-terminal, polybasic region of PrP(C) dictates the efficiency of prion propagation by binding to PrP(Sc).
  Turnbaugh JA, Unterberger U, Saá P, Massignan T, Fluharty BR, Bowman FP, Miller MB, Supattapone S, Biasini E, Harris DA. Turnbaugh JA, et al. J Neurosci. 2012 Jun 27;32(26):8817-30. doi: 10.1523/JNEUROSCI.1103-12.2012. J Neurosci. 2012. PMID: 22745483 Free PMC article.
• Prions.
  Colby DW, Prusiner SB. Colby DW, et al. Cold Spring Harb Perspect Biol. 2011 Jan 1;3(1):a006833. doi: 10.1101/cshperspect.a006833. Cold Spring Harb Perspect Biol. 2011. PMID: 21421910 Free PMC article. Review.
• Molecular biology and pathology of prion strains in sporadic human prion diseases.
  Gambetti P, Cali I, Notari S, Kong Q, Zou WQ, Surewicz WK. Gambetti P, et al. Acta Neuropathol. 2011 Jan;121(1):79-90. doi: 10.1007/s00401-010-0761-3. Epub 2010 Nov 7. Acta Neuropathol. 2011. PMID: 21058033 Free PMC article. Review.

Cited by

• Molecular analysis of the protease-resistant prion protein in scrapie and bovine spongiform encephalopathy transmitted to ovine transgenic and wild-type mice.
  Baron T, Crozet C, Biacabe AG, Philippe S, Verchere J, Bencsik A, Madec JY, Calavas D, Samarut J. Baron T, et al. J Virol. 2004 Jun;78(12):6243-51. doi: 10.1128/JVI.78.12.6243-6251.2004. J Virol. 2004. PMID: 15163717 Free PMC article.
• New insights into early sequential PrPsc accumulation in scrapie infected mouse brain evidenced by the use of streptomycin sulfate.
  Bencsik AA, Leclere E, Perron H, Moussa A. Bencsik AA, et al. Histochem Cell Biol. 2008 May;129(5):643-50. doi: 10.1007/s00418-008-0382-2. Epub 2008 Jan 26. Histochem Cell Biol. 2008. PMID: 18224331
• N-Glycosylation as a Modulator of Protein Conformation and Assembly in Disease.
  Pasala C, Sharma S, Roychowdhury T, Moroni E, Colombo G, Chiosis G. Pasala C, et al. Biomolecules. 2024 Feb 27;14(3):282. doi: 10.3390/biom14030282. Biomolecules. 2024. PMID: 38540703 Free PMC article. Review.
• Modulation of Disordered Proteins with a Focus on Neurodegenerative Diseases and Other Pathologies.
  Martinelli AHS, Lopes FC, John EBO, Carlini CR, Ligabue-Braun R. Martinelli AHS, et al. Int J Mol Sci. 2019 Mar 15;20(6):1322. doi: 10.3390/ijms20061322. Int J Mol Sci. 2019. PMID: 30875980 Free PMC article. Review.
• Prions.
  Prusiner SB. Prusiner SB. Proc Natl Acad Sci U S A. 1998 Nov 10;95(23):13363-83. doi: 10.1073/pnas.95.23.13363. Proc Natl Acad Sci U S A. 1998. PMID: 9811807 Free PMC article. Review.

Publication types

MeSH terms

Substances

LinkOut - more resources

• Full Text Sources

  • Elsevier Science

• Other Literature Sources

  • The Lens - Patent Citations Database

• Research Materials

  • NCI CPTC Antibody Characterization Program