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Results: 1 to 20 of 93

1.

Failure of prion protein oxidative folding guides the formation of toxic transmembrane forms.

Lisa S, Domingo B, Martínez J, Gilch S, Llopis JF, Schätzl HM, Gasset M.

J Biol Chem. 2012 Oct 26;287(44):36693-701. doi: 10.1074/jbc.M112.398776. Epub 2012 Sep 6.

PMID:
22955286
[PubMed - indexed for MEDLINE]
Free PMC Article
2.

Selective processing and metabolism of disease-causing mutant prion proteins.

Ashok A, Hegde RS.

PLoS Pathog. 2009 Jun;5(6):e1000479. doi: 10.1371/journal.ppat.1000479. Epub 2009 Jun 19.

PMID:
19543376
[PubMed - indexed for MEDLINE]
Free PMC Article
3.

Post-translational import of the prion protein into the endoplasmic reticulum interferes with cell viability: a critical role for the putative transmembrane domain.

Heller U, Winklhofer KF, Heske J, Reintjes A, Tatzelt J.

J Biol Chem. 2003 Sep 19;278(38):36139-47. Epub 2003 Jul 8.

PMID:
12853456
[PubMed - indexed for MEDLINE]
Free Article
4.

Prion neurotoxicity: insights from prion protein mutants.

Solomon IH, Schepker JA, Harris DA.

Curr Issues Mol Biol. 2010;12(2):51-61. Epub 2009 Sep 18. Review.

PMID:
19767650
[PubMed - indexed for MEDLINE]
Free Article
5.

Polar substitutions in helix 3 of the prion protein produce transmembrane isoforms that disturb vesicle trafficking.

Sanchez-Garcia J, Arbelaez D, Jensen K, Rincon-Limas DE, Fernandez-Funez P.

Hum Mol Genet. 2013 Nov 1;22(21):4253-66. doi: 10.1093/hmg/ddt276. Epub 2013 Jun 13.

PMID:
23771030
[PubMed - indexed for MEDLINE]
6.

The hydrophobic core region governs mutant prion protein aggregation and intracellular retention.

Biasini E, Tapella L, Restelli E, Pozzoli M, Massignan T, Chiesa R.

Biochem J. 2010 Sep 15;430(3):477-86. doi: 10.1042/BJ20100615.

PMID:
20626348
[PubMed - indexed for MEDLINE]
Free Article
7.

Novel dominant-negative prion protein mutants identified from a randomized library.

Ott D, Taraborrelli C, Aguzzi A.

Protein Eng Des Sel. 2008 Oct;21(10):623-9. doi: 10.1093/protein/gzn042. Epub 2008 Aug 2.

PMID:
18676974
[PubMed - indexed for MEDLINE]
Free Article
8.

A structural overview of the vertebrate prion proteins.

Pastore A, Zagari A.

Prion. 2007 Jul-Sep;1(3):185-97. Epub 2007 Jul 8. Review.

PMID:
19164911
[PubMed - indexed for MEDLINE]
Free PMC Article
9.

Interplays between covalent modifications in the endoplasmic reticulum increase conformational diversity in nascent prion protein.

Orsi A, Sitia R.

Prion. 2007 Oct-Dec;1(4):236-42. Epub 2007 Oct 13.

PMID:
19164910
[PubMed - indexed for MEDLINE]
Free PMC Article
10.

Glycosaminoglycan sulphation affects the seeded misfolding of a mutant prion protein.

Lawson VA, Lumicisi B, Welton J, Machalek D, Gouramanis K, Klemm HM, Stewart JD, Masters CL, Hoke DE, Collins SJ, Hill AF.

PLoS One. 2010 Aug 23;5(8):e12351. doi: 10.1371/journal.pone.0012351.

PMID:
20808809
[PubMed - indexed for MEDLINE]
Free PMC Article
11.

The unfolded state of the murine prion protein and properties of single-point mutants related to human prion diseases.

Gerum C, Schlepckow K, Schwalbe H.

J Mol Biol. 2010 Aug 6;401(1):7-12. doi: 10.1016/j.jmb.2010.06.008. Epub 2010 Jun 10.

PMID:
20541558
[PubMed - indexed for MEDLINE]
12.

Pathogenic mutations in the hydrophobic core of the human prion protein can promote structural instability and misfolding.

van der Kamp MW, Daggett V.

J Mol Biol. 2010 Dec 10;404(4):732-48. doi: 10.1016/j.jmb.2010.09.060. Epub 2010 Oct 7.

PMID:
20932979
[PubMed - indexed for MEDLINE]
Free PMC Article
13.

Familial CJD associated PrP mutants within transmembrane region induced Ctm-PrP retention in ER and triggered apoptosis by ER stress in SH-SY5Y cells.

Wang X, Shi Q, Xu K, Gao C, Chen C, Li XL, Wang GR, Tian C, Han J, Dong XP.

PLoS One. 2011 Jan 27;6(1):e14602. doi: 10.1371/journal.pone.0014602.

PMID:
21298055
[PubMed - indexed for MEDLINE]
Free PMC Article
14.

Prion proteins with pathogenic and protective mutations show similar structure and dynamics.

Bae SH, Legname G, Serban A, Prusiner SB, Wright PE, Dyson HJ.

Biochemistry. 2009 Sep 1;48(34):8120-8. doi: 10.1021/bi900923b.

PMID:
19618915
[PubMed - indexed for MEDLINE]
Free PMC Article
15.

Detergent-resistant membrane domains but not the proteasome are involved in the misfolding of a PrP mutant retained in the endoplasmic reticulum.

Campana V, Sarnataro D, Fasano C, Casanova P, Paladino S, Zurzolo C.

J Cell Sci. 2006 Feb 1;119(Pt 3):433-42.

PMID:
16443748
[PubMed - indexed for MEDLINE]
Free Article
16.

Protein disulfide isomerase regulates endoplasmic reticulum stress and the apoptotic process during prion infection and PrP mutant-induced cytotoxicity.

Wang SB, Shi Q, Xu Y, Xie WL, Zhang J, Tian C, Guo Y, Wang K, Zhang BY, Chen C, Gao C, Dong XP.

PLoS One. 2012;7(6):e38221. doi: 10.1371/journal.pone.0038221. Epub 2012 Jun 7.

PMID:
22685557
[PubMed - indexed for MEDLINE]
Free PMC Article
17.

Mutational analysis of topological determinants in prion protein (PrP) and measurement of transmembrane and cytosolic PrP during prion infection.

Stewart RS, Harris DA.

J Biol Chem. 2003 Nov 14;278(46):45960-8. Epub 2003 Aug 21.

PMID:
12933795
[PubMed - indexed for MEDLINE]
Free Article
18.

The chaperone protein BiP binds to a mutant prion protein and mediates its degradation by the proteasome.

Jin T, Gu Y, Zanusso G, Sy M, Kumar A, Cohen M, Gambetti P, Singh N.

J Biol Chem. 2000 Dec 8;275(49):38699-704.

PMID:
10970892
[PubMed - indexed for MEDLINE]
Free Article
19.

Ligand binding and hydration in protein misfolding: insights from studies of prion and p53 tumor suppressor proteins.

Silva JL, Vieira TC, Gomes MP, Bom AP, Lima LM, Freitas MS, Ishimaru D, Cordeiro Y, Foguel D.

Acc Chem Res. 2010 Feb 16;43(2):271-9. doi: 10.1021/ar900179t. Review.

PMID:
19817406
[PubMed - indexed for MEDLINE]
Free PMC Article
20.

Mutant prion protein expression is associated with an alteration of the Rab GDP dissociation inhibitor alpha (GDI)/Rab11 pathway.

Massignan T, Biasini E, Lauranzano E, Veglianese P, Pignataro M, Fioriti L, Harris DA, Salmona M, Chiesa R, Bonetto V.

Mol Cell Proteomics. 2010 Apr;9(4):611-22. doi: 10.1074/mcp.M900271-MCP200. Epub 2009 Dec 7.

PMID:
19996123
[PubMed - indexed for MEDLINE]
Free PMC Article

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