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Role of prion protein glycosylation in replication of human prions by protein misfolding cyclic amplification.

Camacho MV, Telling G, Kong Q, Gambetti P, Notari S.

Lab Invest. 2019 Nov;99(11):1741-1748. doi: 10.1038/s41374-019-0282-1. Epub 2019 Jun 27.


Gerstmann-Sträussler-Scheinker disease revisited: accumulation of covalently-linked multimers of internal prion protein fragments.

Cracco L, Xiao X, Nemani SK, Lavrich J, Cali I, Ghetti B, Notari S, Surewicz WK, Gambetti P.

Acta Neuropathol Commun. 2019 May 29;7(1):85. doi: 10.1186/s40478-019-0734-2. Erratum in: Acta Neuropathol Commun. 2019 Aug 14;7(1):131.


PMCA-replicated PrPD in urine of vCJD patients maintains infectivity and strain characteristics of brain PrPD: Transmission study.

Cali I, Lavrich J, Moda F, Kofskey D, Nemani SK, Appleby B, Tagliavini F, Soto C, Gambetti P, Notari S.

Sci Rep. 2019 Mar 26;9(1):5191. doi: 10.1038/s41598-019-41694-0.


Publisher Correction: Early preclinical detection of prions in the skin of prion-infected animals.

Wang Z, Manca M, Foutz A, Camacho MV, Raymond GJ, Race B, Orru CD, Yuan J, Shen P, Li B, Lang Y, Dang J, Adornato A, Williams K, Maurer NR, Gambetti P, Xu B, Surewicz W, Petersen RB, Dong X, Appleby BS, Caughey B, Cui L, Kong Q, Zou WQ.

Nat Commun. 2019 Feb 4;10(1):640. doi: 10.1038/s41467-019-08648-6.


Early preclinical detection of prions in the skin of prion-infected animals.

Wang Z, Manca M, Foutz A, Camacho MV, Raymond GJ, Race B, Orru CD, Yuan J, Shen P, Li B, Lang Y, Dang J, Adornato A, Williams K, Maurer NR, Gambetti P, Xu B, Surewicz W, Petersen RB, Dong X, Appleby BS, Caughey B, Cui L, Kong Q, Zou WQ.

Nat Commun. 2019 Jan 16;10(1):247. doi: 10.1038/s41467-018-08130-9. Erratum in: Nat Commun. 2019 Feb 4;10(1):640.


Co-occurrence of chronic traumatic encephalopathy and prion disease.

Nemani SK, Notari S, Cali I, Alvarez VE, Kofskey D, Cohen M, Stern RA, Appleby B, Abrams J, Schonberger L, McKee A, Gambetti P.

Acta Neuropathol Commun. 2018 Dec 18;6(1):140. doi: 10.1186/s40478-018-0643-9.


Variable Protease-Sensitive Prionopathy Transmission to Bank Voles.

Nonno R, Notari S, Di Bari MA, Cali I, Pirisinu L, d'Agostino C, Cracco L, Kofskey D, Vanni I, Lavrich J, Parchi P, Agrimi U, Gambetti P.

Emerg Infect Dis. 2019 Jan;25(1):73-81. doi: 10.3201/eid2501.180807.


Impaired transmissibility of atypical prions from genetic CJDG114V.

Cali I, Mikhail F, Qin K, Gregory C, Solanki A, Martinez MC, Zhao L, Appleby B, Gambetti P, Norstrom E, Mastrianni JA.

Neurol Genet. 2018 Aug 7;4(4):e253. doi: 10.1212/NXG.0000000000000253. eCollection 2018 Aug.


Fatal familial insomnia and sporadic fatal insomnia.

Cracco L, Appleby BS, Gambetti P.

Handb Clin Neurol. 2018;153:271-299. doi: 10.1016/B978-0-444-63945-5.00015-5.


Variably protease-sensitive prionopathy.

Notari S, Appleby BS, Gambetti P.

Handb Clin Neurol. 2018;153:175-190. doi: 10.1016/B978-0-444-63945-5.00010-6.


Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study.

Cali I, Cohen ML, Haik S, Parchi P, Giaccone G, Collins SJ, Kofskey D, Wang H, McLean CA, Brandel JP, Privat N, Sazdovitch V, Duyckaerts C, Kitamoto T, Belay ED, Maddox RA, Tagliavini F, Pocchiari M, Leschek E, Appleby BS, Safar JG, Schonberger LB, Gambetti P.

Acta Neuropathol Commun. 2018 Jan 8;6(1):5. doi: 10.1186/s40478-017-0503-z.


Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) Correlation of Histopathology and MRI in Prion Disease.

Mente KP, O'Donnell JK, Jones SE, Cohen ML, Thompson NR, Bizzi A, Gambetti P, Safar JG, Appleby BS.

Alzheimer Dis Assoc Disord. 2017 Jan-Mar;31(1):1-7. doi: 10.1097/WAD.0000000000000188.


Novel strain properties distinguishing sporadic prion diseases sharing prion protein genotype and prion type.

Cracco L, Notari S, Cali I, Sy MS, Chen SG, Cohen ML, Ghetti B, Appleby BS, Zou WQ, Caughey B, Safar JG, Gambetti P.

Sci Rep. 2017 Jan 16;7:38280. doi: 10.1038/srep38280.


Diagnostic and prognostic value of human prion detection in cerebrospinal fluid.

Foutz A, Appleby BS, Hamlin C, Liu X, Yang S, Cohen Y, Chen W, Blevins J, Fausett C, Wang H, Gambetti P, Zhang S, Hughson A, Tatsuoka C, Schonberger LB, Cohen ML, Caughey B, Safar JG.

Ann Neurol. 2017 Jan;81(1):79-92. doi: 10.1002/ana.24833.


Amyloid fibrils from the N-terminal prion protein fragment are infectious.

Choi JK, Cali I, Surewicz K, Kong Q, Gambetti P, Surewicz WK.

Proc Natl Acad Sci U S A. 2016 Nov 29;113(48):13851-13856. Epub 2016 Nov 14.


A case cluster of variant Creutzfeldt-Jakob disease linked to the Kingdom of Saudi Arabia.

Coulthart MB, Geschwind MD, Qureshi S, Phielipp N, Demarsh A, Abrams JY, Belay E, Gambetti P, Jansen GH, Lang AE, Schonberger LB.

Brain. 2016 Oct;139(Pt 10):2609-2616.


Clinicopathological Correlates in a PRNP P102L Mutation Carrier with Rapidly Progressing Parkinsonism-dystonia.

Umeh CC, Kalakoti P, Greenberg MK, Notari S, Cohen Y, Gambetti P, Oblak AL, Ghetti B, Mari Z.

Mov Disord Clin Pract. 2016 Jul-Aug;3(4):355-358. Epub 2016 Feb 18.


Variants of PLCXD3 are not associated with variant or sporadic Creutzfeldt-Jakob disease in a large international study.

Balendra R, Uphill J, Collinson C, Druyeh R, Adamson G, Hummerich H, Zerr I, Gambetti P, Collinge J, Mead S.

BMC Med Genet. 2016 Apr 7;17:28. doi: 10.1186/s12881-016-0278-2.


Gerstmann-Sträussler-Scheinker disease subtypes efficiently transmit in bank voles as genuine prion diseases.

Pirisinu L, Di Bari MA, D'Agostino C, Marcon S, Riccardi G, Poleggi A, Cohen ML, Appleby BS, Gambetti P, Ghetti B, Agrimi U, Nonno R.

Sci Rep. 2016 Feb 4;6:20443. doi: 10.1038/srep20443.


Quantifying prion disease penetrance using large population control cohorts.

Minikel EV, Vallabh SM, Lek M, Estrada K, Samocha KE, Sathirapongsasuti JF, McLean CY, Tung JY, Yu LP, Gambetti P, Blevins J, Zhang S, Cohen Y, Chen W, Yamada M, Hamaguchi T, Sanjo N, Mizusawa H, Nakamura Y, Kitamoto T, Collins SJ, Boyd A, Will RG, Knight R, Ponto C, Zerr I, Kraus TF, Eigenbrod S, Giese A, Calero M, de Pedro-Cuesta J, Haïk S, Laplanche JL, Bouaziz-Amar E, Brandel JP, Capellari S, Parchi P, Poleggi A, Ladogana A, O'Donnell-Luria AH, Karczewski KJ, Marshall JL, Boehnke M, Laakso M, Mohlke KL, Kähler A, Chambert K, McCarroll S, Sullivan PF, Hultman CM, Purcell SM, Sklar P, van der Lee SJ, Rozemuller A, Jansen C, Hofman A, Kraaij R, van Rooij JG, Ikram MA, Uitterlinden AG, van Duijn CM; Exome Aggregation Consortium (ExAC), Daly MJ, MacArthur DG.

Sci Transl Med. 2016 Jan 20;8(322):322ra9. doi: 10.1126/scitranslmed.aad5169.


Correction: Bank Vole Prion Protein As an Apparently Universal Substrate for RT-QuIC-Based Detection and Discrimination of Prion Strains.

Orrú CD, Groveman BR, Raymond LD, Hughson AG, Nonno R, Zou W, Ghetti B, Gambetti P, Caughey B.

PLoS Pathog. 2015 Aug 18;11(8):e1005117. doi: 10.1371/journal.ppat.1005117. eCollection 2015 Aug. No abstract available.


Inherited mtDNA variations are not strong risk factors in human prion disease.

Hudson G, Uphill J, Hummerich H, Blevins J, Gambetti P, Zerr I, Collinge J, Mead S, Chinnery PF.

Neurobiol Aging. 2015 Oct;36(10):2908.e1-3. doi: 10.1016/j.neurobiolaging.2015.07.005. Epub 2015 Jul 10.


Distinct pathological phenotypes of Creutzfeldt-Jakob disease in recipients of prion-contaminated growth hormone.

Cali I, Miller CJ, Parisi JE, Geschwind MD, Gambetti P, Schonberger LB.

Acta Neuropathol Commun. 2015 Jun 25;3:37. doi: 10.1186/s40478-015-0214-2.


Bank Vole Prion Protein As an Apparently Universal Substrate for RT-QuIC-Based Detection and Discrimination of Prion Strains.

Orrú CD, Groveman BR, Raymond LD, Hughson AG, Nonno R, Zou W, Ghetti B, Gambetti P, Caughey B.

PLoS Pathog. 2015 Jun 18;11(6):e1004983. doi: 10.1371/journal.ppat.1004983. eCollection 2015 Jun. Erratum in: PLoS Pathog. 2015 Aug;11(8):e1005117.


Recent US Case of Variant Creutzfeldt-Jakob Disease-Global Implications.

Maheshwari A, Fischer M, Gambetti P, Parker A, Ram A, Soto C, Concha-Marambio L, Cohen Y, Belay ED, Maddox RA, Mead S, Goodman C, Kass JS, Schonberger LB, Hussein HM.

Emerg Infect Dis. 2015 May;21(5):750-9. doi: 10.3201/eid2105.142017.


Sporadic Creutzfeldt-Jakob disease in a native Puerto Rican patient.

Del Pilar-Morales EA, Cali I, Chapas J, Bertrán-Pasarell J, Puoti G, Gambetti P, Nobo U.

P R Health Sci J. 2015 Mar;34(1):40-3.


Mathematical models for the diffusion magnetic resonance signal abnormality in patients with prion diseases.

Figini M, Alexander DC, Redaelli V, Fasano F, Grisoli M, Baselli G, Gambetti P, Tagliavini F, Bizzi A.

Neuroimage Clin. 2014 Nov 29;7:142-54. doi: 10.1016/j.nicl.2014.11.017. eCollection 2015.


Comparative Study of Prions in Iatrogenic and Sporadic Creutzfeldt-Jakob Disease.

Xiao X, Yuan J, Qing L, Cali I, Mikol J, Delisle MB, Uro-Coste E, Zeng L, Abouelsaad M, Gazgalis D, Martinez MC, Wang GX, Brown P, Ironside JW, Gambetti P, Kong Q, Zou WQ.

J Clin Cell Immunol. 2014 Aug;5(4). pii: 240.


Transmission characteristics of variably protease-sensitive prionopathy.

Notari S, Xiao X, Espinosa JC, Cohen Y, Qing L, Aguilar-Calvo P, Kofskey D, Cali I, Cracco L, Kong Q, Torres JM, Zou W, Gambetti P.

Emerg Infect Dis. 2014 Dec;20(12):2006-14. doi: 10.3201/eid2012.140548.


R47H TREM2 variant increases risk of typical early-onset Alzheimer's disease but not of prion or frontotemporal dementia.

Slattery CF, Beck JA, Harper L, Adamson G, Abdi Z, Uphill J, Campbell T, Druyeh R, Mahoney CJ, Rohrer JD, Kenny J, Lowe J, Leung KK, Barnes J, Clegg SL, Blair M, Nicholas JM, Guerreiro RJ, Rowe JB, Ponto C, Zerr I, Kretzschmar H, Gambetti P, Crutch SJ, Warren JD, Rossor MN, Fox NC, Collinge J, Schott JM, Mead S.

Alzheimers Dement. 2014 Nov;10(6):602-608.e4. doi: 10.1016/j.jalz.2014.05.1751. Epub 2014 Aug 23.


Prions in the urine of patients with variant Creutzfeldt-Jakob disease.

Moda F, Gambetti P, Notari S, Concha-Marambio L, Catania M, Park KW, Maderna E, Suardi S, Haïk S, Brandel JP, Ironside J, Knight R, Tagliavini F, Soto C.

N Engl J Med. 2014 Aug 7;371(6):530-9. doi: 10.1056/NEJMoa1404401.


Concurrent variably protease-sensitive prionopathy and amyotrophic lateral sclerosis.

Cannon A, Bieniek KF, Lin WL, Notari S, Zou WQ, Gambetti P, Pedraza O, Graff-Radford NR, Ferman TJ, Dickson DW.

Acta Neuropathol. 2014 Aug;128(2):313-315. doi: 10.1007/s00401-014-1309-8. Epub 2014 Jun 14. No abstract available.


Variably Protease-sensitive Prionopathy in an Apparent Cognitively Normal 93-Year-Old.

Ghoshal N, Perry A, McKeel D, Schmidt RE, Carter D, Norton J, Zou WQ, Xiao X, Puoti G, Notari S, Gambetti P, Morris JC, Cairns NJ.

Alzheimer Dis Assoc Disord. 2015 Apr-Jun;29(2):173-6. doi: 10.1097/WAD.0000000000000049. No abstract available.


Genetic CJD with a novel E200G mutation in the prion protein gene and comparison with E200K mutation cases.

Kim MO, Cali I, Oehler A, Fong JC, Wong K, See T, Katz JS, Gambetti P, Bettcher BM, Dearmond SJ, Geschwind MD.

Acta Neuropathol Commun. 2013 Dec 12;1:80. doi: 10.1186/2051-5960-1-80.


Thermodynamic stabilization of the folded domain of prion protein inhibits prion infection in vivo.

Kong Q, Mills JL, Kundu B, Li X, Qing L, Surewicz K, Cali I, Huang S, Zheng M, Swietnicki W, Sönnichsen FD, Gambetti P, Surewicz WK.

Cell Rep. 2013 Jul 25;4(2):248-54. doi: 10.1016/j.celrep.2013.06.030. Epub 2013 Jul 18.


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. Print 2013.


Glycoform-selective prion formation in sporadic and familial forms of prion disease.

Xiao X, Yuan J, Haïk S, Cali I, Zhan Y, Moudjou M, Li B, Laplanche JL, Laude H, Langeveld J, Gambetti P, Kitamoto T, Kong Q, Brandel JP, Cobb BA, Petersen RB, Zou WQ.

PLoS One. 2013;8(3):e58786. doi: 10.1371/journal.pone.0058786. Epub 2013 Mar 19.


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.


Creationism and evolutionism in prions.

Gambetti P.

Am J Pathol. 2013 Mar;182(3):623-7. doi: 10.1016/j.ajpath.2012.12.016. Epub 2013 Feb 1. No abstract available.


Prions in variably protease-sensitive prionopathy: an update.

Zou WQ, Gambetti P, Xiao X, Yuan J, Langeveld J, Pirisinu L.

Pathogens. 2013 Jul 5;2(3):457-71. doi: 10.3390/pathogens2030457. Review.


Constant transmission properties of variant Creutzfeldt-Jakob disease in 5 countries.

Diack AB, Ritchie D, Bishop M, Pinion V, Brandel JP, Haik S, Tagliavini F, Van Duijn C, Belay ED, Gambetti P, Schonberger LB, Piccardo P, Will RG, Manson JC.

Emerg Infect Dis. 2012 Oct;18(10):1574-9. doi: 10.3201/eid1810.120792.


Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in Europe and USA.

Parchi P, de Boni L, Saverioni D, Cohen ML, Ferrer I, Gambetti P, Gelpi E, Giaccone G, Hauw JJ, Höftberger R, Ironside JW, Jansen C, Kovacs GG, Rozemuller A, Seilhean D, Tagliavini F, Giese A, Kretzschmar HA.

Acta Neuropathol. 2012 Oct;124(4):517-29. doi: 10.1007/s00401-012-1002-8. Epub 2012 Jun 30.


Sporadic human prion diseases: molecular insights and diagnosis.

Puoti G, Bizzi A, Forloni G, Safar JG, Tagliavini F, Gambetti P.

Lancet Neurol. 2012 Jul;11(7):618-28. doi: 10.1016/S1474-4422(12)70063-7. Review. Erratum in: Lancet Neurol. 2012 Oct;11(10):841.


Assessing prion infectivity of human urine in sporadic Creutzfeldt-Jakob disease.

Notari S, Qing L, Pocchiari M, Dagdanova A, Hatcher K, Dogterom A, Groisman JF, Lumholtz IB, Puopolo M, Lasmezas C, Chen SG, Kong Q, Gambetti P.

Emerg Infect Dis. 2012 Jan;18(1):21-8. doi: 10.3201/eid1801.110589.


Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease.

Chitravas N, Jung RS, Kofskey DM, Blevins JE, Gambetti P, Leigh RJ, Cohen ML.

Ann Neurol. 2011 Sep;70(3):437-44. doi: 10.1002/ana.22454. Epub 2011 Jun 14.


Variably protease-sensitive prionopathy: a novel disease of the prion protein.

Gambetti P, Puoti G, Zou WQ.

J Mol Neurosci. 2011 Nov;45(3):422-4. doi: 10.1007/s12031-011-9543-1. Epub 2011 May 17.


Generation of a new form of human PrP(Sc) in vitro by interspecies transmission from cervid prions.

Barria MA, Telling GC, Gambetti P, Mastrianni JA, Soto C.

J Biol Chem. 2011 Mar 4;286(9):7490-5. doi: 10.1074/jbc.M110.198465. Epub 2011 Jan 5.


Molecular biology and pathology of prion strains in sporadic human prion diseases.

Gambetti P, Cali I, Notari S, Kong Q, Zou WQ, Surewicz WK.

Acta Neuropathol. 2011 Jan;121(1):79-90. doi: 10.1007/s00401-010-0761-3. Epub 2010 Nov 7. Review.


Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein.

Zou WQ, Puoti G, Xiao X, Yuan J, Qing L, Cali I, Shimoji M, Langeveld JP, Castellani R, Notari S, Crain B, Schmidt RE, Geschwind M, Dearmond SJ, Cairns NJ, Dickson D, Honig L, Torres JM, Mastrianni J, Capellari S, Giaccone G, Belay ED, Schonberger LB, Cohen M, Perry G, Kong Q, Parchi P, Tagliavini F, Gambetti P.

Ann Neurol. 2010 Aug;68(2):162-72. doi: 10.1002/ana.22094.


Characterization of the prion protein in human urine.

Dagdanova A, Ilchenko S, Notari S, Yang Q, Obrenovich ME, Hatcher K, McAnulty P, Huang L, Zou W, Kong Q, Gambetti P, Chen SG.

J Biol Chem. 2010 Oct 1;285(40):30489-95. doi: 10.1074/jbc.M110.161794. Epub 2010 Jul 29.

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