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Items: 1 to 20 of 145

1.

Prion protein with an octapeptide insertion has impaired neuroprotective activity in transgenic mice.

Li A, Piccardo P, Barmada SJ, Ghetti B, Harris DA.

EMBO J. 2007 Jun 6;26(11):2777-85. Epub 2007 May 17.

2.

Multiple biochemical similarities between infectious and non-infectious aggregates of a prion protein carrying an octapeptide insertion.

Biasini E, Medrano AZ, Thellung S, Chiesa R, Harris DA.

J Neurochem. 2008 Mar;104(5):1293-308. Epub 2007 Nov 21.

3.

Prion protein with an insertional mutation accumulates on axonal and dendritic plasmalemma and is associated with distinctive ultrastructural changes.

Jeffrey M, Goodsir C, McGovern G, Barmada SJ, Medrano AZ, Harris DA.

Am J Pathol. 2009 Sep;175(3):1208-17. doi: 10.2353/ajpath.2009.090125. Epub 2009 Aug 21.

4.

Neurological illness in transgenic mice expressing a prion protein with an insertional mutation.

Chiesa R, Piccardo P, Ghetti B, Harris DA.

Neuron. 1998 Dec;21(6):1339-51.

5.

The neuropathological phenotype in transgenic mice expressing different prion protein constructs.

DeArmond SJ, Yang SL, Cayetano-Canlas J, Groth D, Prusiner SB.

Philos Trans R Soc Lond B Biol Sci. 1994 Mar 29;343(1306):415-23. Review.

PMID:
7913760
6.

Prion protein transgenes and the neuropathology in prion diseases.

DeArmond SJ, Prusiner SB.

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

PMID:
7767493
7.

A transgenic model of a familial prion disease.

Harris DA, Chiesa R, Drisaldi B, Quaglio E, Migheli A, Piccardo P, Ghetti B.

Arch Virol Suppl. 2000;(16):103-12. Review.

PMID:
11214912
8.

Molecular approaches to mechanisms of prion diseases.

Bratosiewicz-Wasik J, Wasik TJ, Liberski PP.

Folia Neuropathol. 2004;42 Suppl A:33-46. Review.

PMID:
15449458
9.

Accumulation of protease-resistant prion protein (PrP) and apoptosis of cerebellar granule cells in transgenic mice expressing a PrP insertional mutation.

Chiesa R, Drisaldi B, Quaglio E, Migheli A, Piccardo P, Ghetti B, Harris DA.

Proc Natl Acad Sci U S A. 2000 May 9;97(10):5574-9.

10.

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.

11.

Primary myopathy and accumulation of PrPSc-like molecules in peripheral tissues of transgenic mice expressing a prion protein insertional mutation.

Chiesa R, Pestronk A, Schmidt RE, Tourtellotte WG, Ghetti B, Piccardo P, Harris DA.

Neurobiol Dis. 2001 Apr;8(2):279-88.

PMID:
11300723
12.

Molecular distinction between pathogenic and infectious properties of the prion protein.

Chiesa R, Piccardo P, Quaglio E, Drisaldi B, Si-Hoe SL, Takao M, Ghetti B, Harris DA.

J Virol. 2003 Jul;77(13):7611-22.

13.

Protease-sensitive prions with 144-bp insertion mutations.

Xiao X, Cali I, Dong Z, Puoti G, Yuan J, Qing L, Wang H, Kong Q, Gambetti P, Zou WQ.

Aging (Albany NY). 2013 Mar;5(3):155-73.

14.

A murine model of a familial prion disease.

Harris DA, Chiesa R, Drisaldi B, Quaglio E, Migheli A, Piccardo P, Ghetti B.

Clin Lab Med. 2003 Mar;23(1):175-86. Review.

PMID:
12733431
15.

Analysis of the cerebellar proteome in a transgenic mouse model of inherited prion disease reveals preclinical alteration of calcineurin activity.

Biasini E, Massignan T, Fioriti L, Rossi V, Dossena S, Salmona M, Forloni G, Bonetto V, Chiesa R.

Proteomics. 2006 May;6(9):2823-34.

PMID:
16572473
16.

Inherited prion disease caused by the V210I mutation: transmission to transgenic mice.

Mastrianni JA, Capellari S, Telling GC, Han D, Bosque P, Prusiner SB, DeArmond SJ.

Neurology. 2001 Dec 26;57(12):2198-205.

PMID:
11756597
17.

The prion gene complex encoding PrP(C) and Doppel: insights from mutational analysis.

Mastrangelo P, Westaway D.

Gene. 2001 Sep 5;275(1):1-18. Review.

PMID:
11574147
18.

Huntington disease phenocopy is a familial prion disease.

Moore RC, Xiang F, Monaghan J, Han D, Zhang Z, Edström L, Anvret M, Prusiner SB.

Am J Hum Genet. 2001 Dec;69(6):1385-8. Epub 2001 Oct 9.

19.

Phenotypic heterogeneity in inherited prion disease (P102L) is associated with differential propagation of protease-resistant wild-type and mutant prion protein.

Wadsworth JD, Joiner S, Linehan JM, Cooper S, Powell C, Mallinson G, Buckell J, Gowland I, Asante EA, Budka H, Brandner S, Collinge J.

Brain. 2006 Jun;129(Pt 6):1557-69. Epub 2006 Apr 5.

PMID:
16597650
20.

GFP-tagged mutant prion protein forms intra-axonal aggregates in transgenic mice.

Medrano AZ, Barmada SJ, Biasini E, Harris DA.

Neurobiol Dis. 2008 Jul;31(1):20-32. doi: 10.1016/j.nbd.2008.03.006. Epub 2008 Apr 7.

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