23 Publications
2008
Aggregated, wild-type prion protein causes neurological dysfunction and synaptic abnormalities.
Chiesa, RobertoPiccardo, PedroBiasini, EmilianoGhetti, Bernardino and Harris, David A
The Journal of neuroscience : the official journal of the Society for Neuroscience, vol. 28, (no. 49), pp. 13258-67, 2008/Dec/3. | Journal Article
2007
Prion protein with an octapeptide insertion has impaired neuroprotective activity in transgenic mice
Li, AiminLi, A.Piccardo, PedroPiccardo, P.Barmada, S.J.Barmada, Sami JGhetti, B.Ghetti, BernardinoHarris, David A and Harris, D.A.
EMBO Journal, vol. 26, (no. 11), pp. 2777-2785, 2007. | Journal Article
2005
Bax deletion prevents neuronal loss but not neurological symptoms in a transgenic model of inherited prion disease
Chiesa, RobertoPiccardo, PedroDossena, SaraNowoslawski, LisaRoth, KevinGhetti, Bernardino and Harris, David A
Proceedings of the National Academy of Sciences, USA, vol. 102, (no. 1), pp. 238-243, 2005. | Journal Article
2004
GFP-tagged prion protein is correctly localized and functionally active in the brains of transgenic mice
Barmada, Sami JBarmada, S.Piccardo, P.Piccardo, PedroYamaguchi, K.Yamaguchi, KeijiGhetti, BernardinoGhetti, B.Harris, D.A. and Harris, David A
Neurobiology of Disease, vol. 16, (no. 3), pp. 527-537, 2004. | Journal Article
2003
Molecular Distinction between Pathogenic and Infectious Properties of the Prion Protein
Chiesa, R.Piccardo, PedroQuaglio, E.Drisaldi, B.Si-Hoe, SLTakao, M.Ghetti, Bernardino and Harris, David A
Journal of Virology, vol. 77, (no. 13), pp. 7611-7622, 2003. | Journal Article
 
Variable phenotype in a P102L Gerstmann-Sträussler-Scheinker Italian family.
De Michele, GiuseppePocchiari, MaurizioPetraroli, RossellaManfredi, MarioCaneve, GiorgioCoppola, GiovanniCasali, CarloSaccà, FrancescoPiccardo, PedroSalvatore, ElenaBerardelli, AlfredoOrio, MarcelloBarbieri, FabrizioGhetti, Bernardino and Filla, Alessandro
The Canadian Journal of Neurological Sciences. Le Journal Canadien des Sciences Neurologiques, vol. 30, (no. 3), pp. 233-6, 2003/Aug. | Journal Article
2002
A novel mutation (G217D) in the Presenilin 1 gene ( PSEN1) in a Japanese family: presenile dementia and parkinsonism are associated with cotton wool plaques in the cortex and striatum.
Takao, MasakiGhetti, BernardinoHayakawa, IsaoIkeda, EijiFukuuchi, YasuoMiravalle, LeticiaPiccardo, PedroMurrell, Jill RGlazier, Bradley S and Koto, Atsuo
Acta neuropathologica, vol. 104, (no. 2), pp. 155-70, 2002/Aug. | Journal Article
 
Association between conformational mutations in neuroserpin and onset and severity of dementia.
Davis, Richard LShrimpton, Antony ECarrell, RobinLomas, DavidGerhard, LieselotteBaumann, BrunoLawrence, Daniel AYepes, ManuelKim, Tai SGhetti, BernardinoPiccardo, PedroTakao, MasakiLacbawan, Felicitas LMuenke, MaximilianSifers, Richard NBradshaw, Charles BKent, Paul FCollins, George HLarocca, Daria and Holohan, Peter D
Lancet, vol. 359, (no. 9325), pp. 2242-7, 2002/Jun/29. | Journal Article
2001
A 7-kDa prion protein (PrP) fragment, an integral component of the PrP region required for infectivity, is the major amyloid protein in Gerstmann-Sträussler-Scheinker disease A117V.
Tagliavini, FLievens, P MTranchant, CWarter, J MMohr, MGiaccone, GPerini, FRossi, GSalmona, MPiccardo, PedroGhetti, BernardinoBeavis, R CBugiani, OFrangione, Blas and Prelli, F
The Journal of biological chemistry, vol. 276, (no. 8), pp. 6009-15, 2001/Feb/23. | Journal Article
 
Biochemical characterization of a neuroserpin variant associated with hereditary dementia.
Yazaki, MLiepnieks, J JMurrell, Jill RTakao, MGuenther, BPiccardo, PedroFarlow, Martin RGhetti, Bernardino and Benson, Merrill D
The American journal of pathology, vol. 158, (no. 1), pp. 227-33, 2001/Jan. | Journal Article
 
Ectopic White Matter Neurons, a Developmental Abnormality That May Be Caused by the PSEN1 S169L Mutation in a Case of Familial AD with Myoclonus and Seizures
Takao, M.Ghetti, BernardinoMurrell, Jill RUnverzagt, FrederickGiaccone, G.Tagliavini, F.Bugiani, O.Piccardo, PedroHulette, Christine MCrain, Barbara JFarlow, Martin R and Heyman, Albert
Journal of Neuropathology & Experimental Neurology, vol. 60, (no. 12), pp. 1137-1152, 2001. | Journal Article
 
Prion proteins with different conformations accumulate in Gerstmann-Sträussler-Scheinker disease caused by A117V and F198S mutations.
Piccardo, PedroPiccardo, PedroPiccardo, PedroPiccardo, PedroLiepnieks, J JLiepnieks, Juris JLiepnieks, Juris JLiepnieks, Juris JWilliam, AWilliam, AlbertWilliam, AlbertWilliam, AlbertDlouhy, Stephen RDlouhy, Stephen RDlouhy, S RDlouhy, Stephen RFarlow, Martin RFarlow, M RFarlow, Martin RFarlow, Martin RYoung, KatherineYoung, KYoung, KatherineYoung, KatherineNochlin, DavidNochlin, DavidNochlin, DavidNochlin, DavidBird, Thomas DBird, Thomas DBird, Thomas DBird, T DNixon, Randal RNixon, Randal RNixon, Randal RNixon, R RBall, Melvyn JBall, M JBall, Melvyn JBall, Melvyn JDeCarli, CharlesDeCarli, CDeCarli, CharlesDeCarli, CharlesBugiani, OBugiani, OrsoBugiani, OrsoBugiani, OrsoTagliavini, FabrizioTagliavini, FabrizioTagliavini, FTagliavini, FabrizioBenson, Merrill DBenson, Merrill DBenson, Merrill DBenson, M DGhetti, BernardinoGhetti, BGhetti, Bernardino and Ghetti, Bernardino
The American journal of pathology, vol. 158, (no. 6), pp. 2201-7, 2001/Jun. | Journal Article
2000
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, PedroGhetti, Bernardino and Harris, David A
Proceedings of the National Academy of Sciences, USA, vol. 97, (no. 10), pp. 5580-5585, 2000. | Journal Article
 
Accumulation of protease-resistant prion protein (PrP) and apoptosis of cerebellar granule cells in transgenic mice expressing a PrP insertional mutation.
Chiesa, RDrisaldi, BQuaglio, EMigheli, APiccardo, PedroGhetti, Bernardino and Harris, David A
Proceedings of the National Academy of Sciences of the United States of America, vol. 97, (no. 10), pp. 5574-9, 2000/May/9. | Journal Article
 
Neuroserpin Mutation S52R Causes Neuroserpin Accumulation in Neurons and Is Associated with Progressive Myoclonus Epilepsy
Takao, M.Benson, Merrill DMurrell, Jill RYazaki, M.Piccardo, PedroUnverzagt, FrederickDavis, Robin LHolohan, PDLawrence, Daniel ARichardson, R.Farlow, Martin R and Ghetti, Bernardino
Journal of Neuropathology & Experimental Neurology, vol. 59, (no. 12), pp. 1070-1086, 2000. | Journal Article
1998
Endogenous proteolytic cleavage of normal and disease-associated isoforms of the human prion protein in neural and non-neural tissues.
Jiménez-Huete, ALievens, P MVidal, Ruben GPiccardo, PedroGhetti, BernardinoTagliavini, FFrangione, Blas and Prelli, F
The American journal of pathology, vol. 153, (no. 5), pp. 1561-72, 1998/Nov. | Journal Article
 
Neurological Illness in Transgenic Mice Expressing a Prion Protein with an Insertional Mutation
Chiesa, R.Piccardo, PedroGhetti, Bernardino and Harris, David A
Neuron, vol. 21, (no. 6), pp. 1339-1351, 1998. | Journal Article
 
Phenotypic variability of Gerstmann-Sträussler-Scheinker disease is associated with prion protein heterogeneity.
Piccardo, PedroDlouhy, Stephen RLievens, P MYoung, KBird, Thomas DNochlin, DavidDickson, Dennis WVinters, Harry VZimmerman, T RMackenzie, IanKish, S JAng, L CDe Carli, CPocchiari, MBrown, PGibbs, C JGajdusek, D CBugiani, OIronside, J. WTagliavini, F and Ghetti, Bernardino
Journal of Neuropathology and Experimental Neurology, vol. 57, (no. 10), pp. 979-88, 1998/Oct. | Journal Article
1996
Prion protein hereditary amyloidosis: Parenchymal and vascular
Ghetti, BernardinoPiccardo, PedroFrangione, BlasBugiani, O.Giaccone, G.Young, K.Prelli, F.Farlow, MRDlouhy, S. R and Tagliavini, F.
Seminars in Virology, vol. 7, (no. 3), pp. 189-200, 1996. | Journal Article
 
Proteinase-K-resistant prion protein isoforms in Gerstmann-Sträussler-Scheinker disease (Indiana kindred).
Piccardo, PedroSeiler, CDlouhy, Stephen RYoung, KFarlow, Martin RPrelli, FFrangione, BlasBugiani, OTagliavini, F and Ghetti, Bernardino
Journal of neuropathology and experimental neurology, vol. 55, (no. 11), pp. 1157-63, 1996/Nov. | Journal Article
 
Vascular variant of prion protein cerebral amyloidosis with tau-positive neurofibrillary tangles: the phenotype of the stop codon 145 mutation in PRNP.
Ghetti, BernardinoPiccardo, PedroSpillantini, Maria GIchimiya, YPorro, MPerini, FKitamoto, TetsuyukiTateishi, JSeiler, CFrangione, BlasBugiani, OGiaccone, GPrelli, FGoedert, MDlouhy, Stephen R and Tagliavini, F
Proceedings of the National Academy of Sciences of the United States of America, vol. 93, (no. 2), pp. 744-8, 1996/Jan/23. | Journal Article
1995
Gerstmann-Sträussler-Scheinker disease (PRNP P102L): amyloid deposits are best recognized by antibodies directed to epitopes in PrP region 90-165.
Piccardo, PedroGhetti, BernardinoDickson, Dennis WVinters, H VGiaccone, GBugiani, OTagliavini, FYoung, KDlouhy, S R and Seiler, C
Journal of Neuropathology and Experimental Neurology, vol. 54, (no. 6), pp. 790-801, 1995/Nov. | Journal Article
 
Gerstmann-Sträussler-Scheinker disease with mutation at codon 102 and methionine at codon 129 of PRNP in previously unreported patients.
Young, KJones, C KPiccardo, PedroLazzarini, AGolbe, Lawrence IZimmerman, T RDickson, Dennis WMcLachlan, D CSt George-Hyslop, P and Lennox, A
Neurology, vol. 45, (no. 6), pp. 1127-34, 1995/Jun. | Journal Article