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Mutant PrPSc conformers induced by a synthetic peptide and several prion strains.

Tremblay P, Ball HL, Kaneko K, Groth D, Hegde RS, Cohen FE, DeArmond SJ, Prusiner SB, Safar JG.

J Virol. 2004 Feb;78(4):2088-99.


Genetic and infectious prion diseases.

Prusiner SB.

Arch Neurol. 1993 Nov;50(11):1129-53. Review.


Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein.

Hsiao KK, Groth D, Scott M, Yang SL, Serban H, Rapp D, Foster D, Torchia M, Dearmond SJ, Prusiner SB.

Proc Natl Acad Sci U S A. 1994 Sep 13;91(19):9126-30.


Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice.

Telling GC, Haga T, Torchia M, Tremblay P, DeArmond SJ, Prusiner SB.

Genes Dev. 1996 Jul 15;10(14):1736-50.


A Drosophila model of GSS syndrome suggests defects in active zones are responsible for pathogenesis of GSS syndrome.

Choi JK, Jeon YC, Lee DW, Oh JM, Lee HP, Jeong BH, Carp RI, Koh YH, Kim YS.

Hum Mol Genet. 2010 Nov 15;19(22):4474-89. doi: 10.1093/hmg/ddq379.


Inherited prion disease A117V is not simply a proteinopathy but produces prions transmissible to transgenic mice expressing homologous prion protein.

Asante EA, Linehan JM, Smidak M, Tomlinson A, Grimshaw A, Jeelani A, Jakubcova T, Hamdan S, Powell C, Brandner S, Wadsworth JD, Collinge J.

PLoS Pathog. 2013;9(9):e1003643. doi: 10.1371/journal.ppat.1003643.


Molecular biology and genetics of prion diseases.

Prusiner SB.

Philos Trans R Soc Lond B Biol Sci. 1994 Mar 29;343(1306):447-63. Review.


Prion encephalopathies of animals and humans.

Prusiner SB.

Dev Biol Stand. 1993;80:31-44. Review.


Gerstmann-Sträussler-Scheinker disease and "anchorless prion protein" mice share prion conformational properties diverging from sporadic Creutzfeldt-Jakob disease.

Zanusso G, Fiorini M, Ferrari S, Meade-White K, Barbieri I, Brocchi E, Ghetti B, Monaco S.

J Biol Chem. 2014 Feb 21;289(8):4870-81. doi: 10.1074/jbc.M113.531335.


Accelerated accumulation of misfolded prion protein and spongiform degeneration in a Drosophila model of Gerstmann-Sträussler-Scheinker syndrome.

Gavin BA, Dolph MJ, Deleault NR, Geoghegan JC, Khurana V, Feany MB, Dolph PJ, Supattapone S.

J Neurosci. 2006 Nov 29;26(48):12408-14.


Allelic origin of protease-sensitive and protease-resistant prion protein isoforms in Gerstmann-Sträussler-Scheinker disease with the P102L mutation.

Monaco S, Fiorini M, Farinazzo A, Ferrari S, Gelati M, Piccardo P, Zanusso G, Ghetti B.

PLoS One. 2012;7(2):e32382. doi: 10.1371/journal.pone.0032382.


Pathogenic mutations within the hydrophobic domain of the prion protein lead to the formation of protease-sensitive prion species with increased lethality.

Coleman BM, Harrison CF, Guo B, Masters CL, Barnham KJ, Lawson VA, Hill AF.

J Virol. 2014 Mar;88(5):2690-703. doi: 10.1128/JVI.02720-13.


Small ruminant nor98 prions share biochemical features with human gerstmann-sträussler-scheinker disease and variably protease-sensitive prionopathy.

Pirisinu L, Nonno R, Esposito E, Benestad SL, Gambetti P, Agrimi U, Zou WQ.

PLoS One. 2013 Jun 24;8(6):e66405. doi: 10.1371/journal.pone.0066405.


Prion protein transgenes and the neuropathology in prion diseases.

DeArmond SJ, Prusiner SB.

Brain Pathol. 1995 Jan;5(1):77-89. Review.


Prion seeding activities of mouse scrapie strains with divergent PrPSc protease sensitivities and amyloid plaque content using RT-QuIC and eQuIC.

Vascellari S, Orrù CD, Hughson AG, King D, Barron R, Wilham JM, Baron GS, Race B, Pani A, Caughey B.

PLoS One. 2012;7(11):e48969. doi: 10.1371/journal.pone.0048969.


Transmission Properties of Human PrP 102L Prions Challenge the Relevance of Mouse Models of GSS.

Asante EA, Grimshaw A, Smidak M, Jakubcova T, Tomlinson A, Jeelani A, Hamdan S, Powell C, Joiner S, Linehan JM, Brandner S, Wadsworth JD, Collinge J.

PLoS Pathog. 2015 Jul 2;11(7):e1004953. doi: 10.1371/journal.ppat.1004953.


Sc237 hamster PrPSc and Sc237-derived mouse PrPSc generated by interspecies in vitro amplification exhibit distinct pathological and biochemical properties in tga20 transgenic mice.

Yoshioka M, Imamura M, Okada H, Shimozaki N, Murayama Y, Yokoyama T, Mohri S.

Microbiol Immunol. 2011 May;55(5):331-40. doi: 10.1111/j.1348-0421.2011.00328.x.


Molecular biology and pathogenesis of prion diseases.

Prusiner SB.

Trends Biochem Sci. 1996 Dec;21(12):482-7. Review.


Mouse prion protein (PrP) segment 100 to 104 regulates conversion of PrP(C) to PrP(Sc) in prion-infected neuroblastoma cells.

Hara H, Okemoto-Nakamura Y, Shinkai-Ouchi F, Hanada K, Yamakawa Y, Hagiwara K.

J Virol. 2012 May;86(10):5626-36. doi: 10.1128/JVI.06606-11.


Dominant-negative inhibition of prion replication in transgenic mice.

Perrier V, Kaneko K, Safar J, Vergara J, Tremblay P, DeArmond SJ, Cohen FE, Prusiner SB, Wallace AC.

Proc Natl Acad Sci U S A. 2002 Oct 1;99(20):13079-84.

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