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Results: 1 to 20 of 123

1.

Epitope scanning indicates structural differences in brain-derived monomeric and aggregated mutant prion proteins related to genetic prion diseases.

Tapella L, Stravalaci M, Bastone A, Biasini E, Gobbi M, Chiesa R.

Biochem J. 2013 Sep 15;454(3):417-25. doi: 10.1042/BJ20130563.

PMID:
23808898
[PubMed - indexed for MEDLINE]
Free Article
2.

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. Epub 2014 Jan 7.

PMID:
24398683
[PubMed - indexed for MEDLINE]
3.

A New Transgenic Mouse Model of Gerstmann-Straussler-Scheinker Syndrome Caused by the A117V Mutation of PRNP.

Yang W, Cook J, Rassbach B, Lemus A, DeArmond SJ, Mastrianni JA.

J Neurosci. 2009 Aug 12;29(32):10072-80. doi: 10.1523/JNEUROSCI.2542-09.2009.

PMID:
19675240
[PubMed - indexed for MEDLINE]
Free PMC Article
4.

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. Epub 2013 Sep 26.

PMID:
24086135
[PubMed - indexed for MEDLINE]
Free PMC Article
5.

Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Sträussler syndrome.

Kitamoto T, Ohta M, Doh-ura K, Hitoshi S, Terao Y, Tateishi J.

Biochem Biophys Res Commun. 1993 Mar 15;191(2):709-14.

PMID:
8461023
[PubMed - indexed for MEDLINE]
6.

[Genetic background of human prion diseases].

Kovács GG.

Ideggyogy Sz. 2007 Nov 30;60(11-12):438-46. Review. Hungarian.

PMID:
18198790
[PubMed - indexed for MEDLINE]
7.

Different patterns of truncated prion protein fragments correlate with distinct phenotypes in P102L Gerstmann-Sträussler-Scheinker disease.

Parchi P, Chen SG, Brown P, Zou W, Capellari S, Budka H, Hainfellner J, Reyes PF, Golden GT, Hauw JJ, Gajdusek DC, Gambetti P.

Proc Natl Acad Sci U S A. 1998 Jul 7;95(14):8322-7.

PMID:
9653185
[PubMed - indexed for MEDLINE]
Free PMC Article
8.

An antibody to the aggregated synthetic prion protein peptide (PrP106-126) selectively recognizes disease-associated prion protein (PrP) from human brain specimens.

Jones M, Wight D, McLoughlin V, Norrby K, Ironside JW, Connolly JG, Farquhar CF, MacGregor IR, Head MW.

Brain Pathol. 2009 Apr;19(2):293-302. doi: 10.1111/j.1750-3639.2008.00181.x. Epub 2008 May 26.

PMID:
18507665
[PubMed - indexed for MEDLINE]
9.

Familial mutations and the thermodynamic stability of the recombinant human prion protein.

Swietnicki W, Petersen RB, Gambetti P, Surewicz WK.

J Biol Chem. 1998 Nov 20;273(47):31048-52.

PMID:
9813003
[PubMed - indexed for MEDLINE]
Free Article
10.

Insoluble wild-type and protease-resistant mutant prion protein in brains of patients with inherited prion disease.

Gabizon R, Telling G, Meiner Z, Halimi M, Kahana I, Prusiner SB.

Nat Med. 1996 Jan;2(1):59-64.

PMID:
8564843
[PubMed - indexed for MEDLINE]
11.

The prion diseases.

Brown K, Mastrianni JA.

J Geriatr Psychiatry Neurol. 2010 Dec;23(4):277-98. doi: 10.1177/0891988710383576. Epub 2010 Oct 11. Review.

PMID:
20938044
[PubMed - indexed for MEDLINE]
12.

Prion encephalopathies of animals and humans.

Prusiner SB.

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

PMID:
8270114
[PubMed - indexed for MEDLINE]
13.

Aggresome formation by mutant prion proteins: the unfolding role of proteasomes in familial prion disorders.

Mishra RS, Bose S, Gu Y, Li R, Singh N.

J Alzheimers Dis. 2003 Feb;5(1):15-23.

PMID:
12590162
[PubMed - indexed for MEDLINE]
14.

Genetic Creutzfeldt-Jakob disease and fatal familial insomnia: insights into phenotypic variability and disease pathogenesis.

Capellari S, Strammiello R, Saverioni D, Kretzschmar H, Parchi P.

Acta Neuropathol. 2011 Jan;121(1):21-37. doi: 10.1007/s00401-010-0760-4. Epub 2010 Oct 27. Review.

PMID:
20978903
[PubMed - indexed for MEDLINE]
15.

Substitutions at residue 211 in the prion protein drive a switch between CJD and GSS syndrome, a new mechanism governing inherited neurodegenerative disorders.

Peoc'h K, Levavasseur E, Delmont E, De Simone A, Laffont-Proust I, Privat N, Chebaro Y, Chapuis C, Bedoucha P, Brandel JP, Laquerriere A, Kemeny JL, Hauw JJ, Borg M, Rezaei H, Derreumaux P, Laplanche JL, Haïk S.

Hum Mol Genet. 2012 Dec 15;21(26):5417-28. doi: 10.1093/hmg/dds377. Epub 2012 Sep 10. Erratum in: Hum Mol Genet. 2014 Apr 15;23(8):2234.

PMID:
22965875
[PubMed - indexed for MEDLINE]
Free Article
16.

Genetic and infectious prion diseases.

Prusiner SB.

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

PMID:
8105771
[PubMed - indexed for MEDLINE]
17.

[Molecular genetics in Creutzfeldt-Jakob disease].

Kitamoto T.

Rinsho Shinkeigaku. 1994 Dec;34(12):1222-3. Review. Japanese.

PMID:
7774117
[PubMed - indexed for MEDLINE]
18.

The primary structure of the prion protein influences the distribution of abnormal prion protein in the central nervous system.

Kitamoto T, Doh-ura K, Muramoto T, Miyazono M, Tateishi J.

Am J Pathol. 1992 Aug;141(2):271-7.

PMID:
1353945
[PubMed - indexed for MEDLINE]
Free PMC Article
19.

Analyses of Gerstmann-Straussler syndrome with 102Leu219Lys using monoclonal antibodies that specifically detect human prion protein with 219Glu.

Muramoto T, Tanaka T, Kitamoto N, Sano C, Hayashi Y, Kutomi T, Yutani C, Kitamoto T.

Neurosci Lett. 2000 Jul 21;288(3):179-82.

PMID:
10889337
[PubMed - indexed for MEDLINE]
20.

Proteinase-K-resistant prion protein isoforms in Gerstmann-Sträussler-Scheinker disease (Indiana kindred).

Piccardo P, Seiler C, Dlouhy SR, Young K, Farlow MR, Prelli F, Frangione B, Bugiani O, Tagliavini F, Ghetti B.

J Neuropathol Exp Neurol. 1996 Nov;55(11):1157-63.

PMID:
8939199
[PubMed - indexed for MEDLINE]

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