Pierluigi Gambetti

Pierluigi Gambetti

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Pierluigi Gambetti

Publications by authors named "Pierluigi Gambetti"

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

Lab Invest 2019 Jun 27. Epub 2019 Jun 27.

Department of Pathology, Case Western Reserve University, Cleveland, OH, USA.

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http://dx.doi.org/10.1038/s41374-019-0282-1DOI Listing
June 2019

Co-occurrence of chronic traumatic encephalopathy and prion disease.

Acta Neuropathol Commun 2018 12 18;6(1):140. Epub 2018 Dec 18.

Department of Pathology, Case Western Reserve University, School of Medicine, Cleveland, OH, 44106, USA.

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http://dx.doi.org/10.1186/s40478-018-0643-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6299534PMC
December 2018

Variably protease-sensitive prionopathy.

Handb Clin Neurol 2018 ;153:175-190

Department of Pathology, Case Western Reserve University, Cleveland, OH, United States. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/B97804446394550
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http://dx.doi.org/10.1016/B978-0-444-63945-5.00010-6DOI Listing
September 2018

Fatal familial insomnia and sporadic fatal insomnia.

Handb Clin Neurol 2018 ;153:271-299

Department of Pathology, Case Western Reserve University, Cleveland, OH, United States. Electronic address:

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http://dx.doi.org/10.1016/B978-0-444-63945-5.00015-5DOI Listing
September 2018

Impaired transmissibility of atypical prions from genetic CJD.

Neurol Genet 2018 Aug 7;4(4):e253. Epub 2018 Aug 7.

Department of Pathology (I.C., M.C.M., P.G.), Case Western University, Cleveland, OH; Department of Neurology (K.Q., F.M., C.G., A.S., L.Z., J.A.M.), University of Chicago; and Department of Biological Sciences (E.N.), DePaul University, Chicago, IL.

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http://dx.doi.org/10.1212/NXG.0000000000000253DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6089695PMC
August 2018

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

Alzheimer Dis Assoc Disord 2017 Jan-Mar;31(1):1-7

Departments of *Neurology ‡Diagnostic Radiology ¶Quantitative Health Sciences, Cleveland Clinic Departments of †Radiology §Pathology **Neurology, University Hospitals Case Medical Center ∥National Prion Disease Pathology Surveillance Center #Department of Neuroradiology, Humanitas Research Hospital IRCCS, Milano, Italy.

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http://dx.doi.org/10.1097/WAD.0000000000000188DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5322151PMC
November 2017

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

Proc Natl Acad Sci U S A 2016 11 14;113(48):13851-13856. Epub 2016 Nov 14.

Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, OH 44106;

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http://dx.doi.org/10.1073/pnas.1610716113DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5137684PMC
November 2016

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

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

6 Division of High Consequence Pathogens and Pathology, National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, GA 30333, USA.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5082737PMC
http://dx.doi.org/10.1093/brain/aww206DOI Listing
October 2016

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

BMC Med Genet 2016 Apr 7;17:28. Epub 2016 Apr 7.

MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, Queen Square, London, WC1N 3BG, UK.

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http://dx.doi.org/10.1186/s12881-016-0278-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4823897PMC
April 2016

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

Alzheimer Dis Assoc Disord 2015 Apr-Jun;29(2):173-6

Departments of *Neurology, Charles F. and Joanne Knight Alzheimer's Disease Research Center †Pathology & Immunology, Washington University School of Medicine, St Louis, MO ‡Department of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH.

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http://dx.doi.org/10.1097/WAD.0000000000000049DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4237693PMC
February 2016

Quantifying prion disease penetrance using large population control cohorts.

Sci Transl Med 2016 Jan;8(322):322ra9

Program in Medical and Population Genetics, Broad Institute of Massachusetts Institute of Technology (MIT) and Harvard, Cambridge, MA 02142, USA. Analytical and Translational Genetics Unit, Massachusetts General Hospital, Boston, MA 02114, USA.

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http://dx.doi.org/10.1126/scitranslmed.aad5169DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4774245PMC
January 2016

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

Neurobiol Aging 2015 Oct 10;36(10):2908.e1-3. Epub 2015 Jul 10.

Wellcome Trust Centre for Mitochondrial Research, Institute of Genetic Medicine, Newcastle University, UK.

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http://dx.doi.org/10.1016/j.neurobiolaging.2015.07.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6542657PMC
October 2015

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

PLoS Pathog 2015 Jun 18;11(6):e1004983. Epub 2015 Jun 18.

Laboratory of Persistent Viral Diseases, Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH), Hamilton, Montana, United States of America.

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http://dx.doi.org/10.1371/journal.ppat.1004983DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4472236PMC
June 2015

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

Acta Neuropathol Commun 2015 Jun 25;3:37. Epub 2015 Jun 25.

Division of High Consequence Pathogens and Pathology, National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, GA, USA.

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http://dx.doi.org/10.1186/s40478-015-0214-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4479081PMC
June 2015

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

N Engl J Med 2014 Aug;371(6):530-9

From the Mitchell Center for Research in Alzheimer's Disease and Related Brain Disorders, University of Texas Medical School at Houston, Houston (F.M., L.C.-M., K.-W.P., C.S.); Foundation Carlo Besta Neurologic Institute, Milan (F.M., M.C., E.M., S.S., F.T.); National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland (P.G., S.N.); Universidad de los Andes, Facultad de Medicina, Santiago, Chile (L.C.-M.); Assistance Publique-Hôpitaux de Paris, Cellule Nationale de Référence des Maladies de Creutzfeldt-Jakob, Groupe Hospitalier Pitié-Salpêtrière, INSERM Unité 1127, Université Pierre et Marie Curie-Paris 6, and Centre Nationale de la Recherche Scientifique, Unité Mixte de Recherche - all in Paris (S.H., J.-P.B.); and the National CJD Research and Surveillance Unit, Western General Hospital, University of Edinburgh, Edinburgh (J.I., R.K.).

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http://dx.doi.org/10.1056/NEJMoa1404401DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4162740PMC
August 2014

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

J Clin Cell Immunol 2014 Aug;5(4)

Department of Pathology and National Prion Disease, Pathology Surveillance Center, Case Western Reserve University, 2085 Adelbert Road, Cleveland, Ohio 44106, USA ; Department of Neurology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, Ohio 44106, USA ; National Center for Regenerative Medicine, Case Western Reserve University, 2085 Adelbert Road, Cleveland, Ohio 44106, USA ; The First Affiliated Hospital, Nanchang University, Nanchang, Jiangxi Province, The People's Republic of China.

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http://dx.doi.org/10.4172/2155-9899.1000240DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4240320PMC
August 2014

Prions in variably protease-sensitive prionopathy: an update.

Pathogens 2013 Jul 5;2(3):457-71. Epub 2013 Jul 5.

Department of Veterinary Public Health and Food Safety, Istituto Superiore di Sanità, Viale Regina Elena 299 00161, Rome, Italy.

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http://dx.doi.org/10.3390/pathogens2030457DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4235694PMC
July 2013

Creationism and evolutionism in prions.

Am J Pathol 2013 Mar 1;182(3):623-7. Epub 2013 Feb 1.

Department of Pathology, Case Western Reserve University, Cleveland, Ohio 44060, USA.

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http://dx.doi.org/10.1016/j.ajpath.2012.12.016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3590995PMC
March 2013

Protease-sensitive prions with 144-bp insertion mutations.

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

Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3629288PMC
http://dx.doi.org/10.18632/aging.100543DOI Listing
March 2013

Sporadic human prion diseases: molecular insights and diagnosis.

Lancet Neurol 2012 Jul;11(7):618-28

Division of Neurology, Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy.

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http://dx.doi.org/10.1016/S1474-4422(12)70063-7DOI Listing
July 2012

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

J Mol Neurosci 2011 Nov 17;45(3):422-4. Epub 2011 May 17.

Department of Pathology, National Prion Disease Pathology Surveillance Center, School of Medicine, Case Western Reserve University, Cleveland, OH 44106, USA.

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http://dx.doi.org/10.1007/s12031-011-9543-1DOI Listing
November 2011

Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease.

Ann Neurol 2011 Sep 14;70(3):437-44. Epub 2011 Jun 14.

Department of Neurology, University Hospitals Case Medical Center, Cleveland, OH 44106, USA.

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http://dx.doi.org/10.1002/ana.22454DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3170496PMC
September 2011

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

J Biol Chem 2011 Mar 5;286(9):7490-5. Epub 2011 Jan 5.

Mitchell Center for Alzheimer's Disease and Related Brain Disorders, Department of Neurology, University of Texas Medical School at Houston, Houston, Texas 77030, USA.

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http://dx.doi.org/10.1074/jbc.M110.198465DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3045004PMC
March 2011

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

Acta Neuropathol 2011 Jan 7;121(1):79-90. Epub 2010 Nov 7.

Department of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH, 44106, USA.

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http://dx.doi.org/10.1007/s00401-010-0761-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3077936PMC
January 2011

Characterization of the prion protein in human urine.

J Biol Chem 2010 Oct 29;285(40):30489-95. Epub 2010 Jul 29.

Department of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA.

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http://dx.doi.org/10.1074/jbc.M110.161794DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2945542PMC
October 2010

The role of glycophosphatidylinositol anchor in the amplification of the scrapie isoform of prion protein in vitro.

FEBS Lett 2009 Nov 23;583(22):3671-5. Epub 2009 Oct 23.

Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, OH 44106, USA.

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http://dx.doi.org/10.1016/j.febslet.2009.10.049DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2856614PMC
November 2009

Codistribution of amyloid beta plaques and spongiform degeneration in familial Creutzfeldt-Jakob disease with the E200K-129M haplotype.

Arch Neurol 2009 Oct;66(10):1240-6

Department of Neurology and Alzheimer's Disease Research Center, Washington University School of Medicine, 4488 Forest Park Ave, Ste 101, St Louis, MO 63108, USA.

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http://dx.doi.org/10.1001/archneurol.2009.224DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2796207PMC
October 2009

Cell-free propagation of prion strains.

EMBO J 2008 Oct 18;27(19):2557-66. Epub 2008 Sep 18.

Department of Neurology, University of Texas Medical Branch, Galveston, TX 77555, USA.

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http://dx.doi.org/10.1038/emboj.2008.181DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2567411PMC
October 2008

Sporadic fatal insomnia masquerading as a paraneoplastic cerebellar syndrome.

Arch Neurol 2008 Jul;65(7):971-3

Neuroimmunology Unit, Department of Neurology, University of Rochester Medical Center, 601 Elmwood Ave, Box 605, Rochester, NY 14642, USA.

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http://dx.doi.org/10.1001/archneur.65.7.971DOI Listing
July 2008

A refined method for molecular typing reveals that co-occurrence of PrP(Sc) types in Creutzfeldt-Jakob disease is not the rule.

Lab Invest 2007 Nov 24;87(11):1103-12. Epub 2007 Sep 24.

Dipartimento di Scienze Neurologiche, Università di Bologna, Bologna, Italy.

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http://dx.doi.org/10.1038/labinvest.3700676DOI Listing
November 2007

Washington statewide pathology surveillance for prion disease.

Ann Neurol 2007 Apr;61(4):371-2

Department of Pathology, University of Washington, Seattle, WA 98104, USA.

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http://dx.doi.org/10.1002/ana.21096DOI Listing
April 2007

Insoluble aggregates and protease-resistant conformers of prion protein in uninfected human brains.

J Biol Chem 2006 Nov 20;281(46):34848-58. Epub 2006 Sep 20.

Department of Pathology and National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio 44106, USA.

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http://dx.doi.org/10.1074/jbc.M602238200DOI Listing
November 2006

Soluble amyloid-beta in the brain: the scarlet pimpernel.

J Alzheimers Dis 2006 ;9(3 Suppl):127-32

University of Genova, Institute of Neurology, Via De Toni 5, 16132 Genova, Italy.

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October 2006

Classification of sporadic Creutzfeldt-Jakob disease revisited.

Brain 2006 Sep;129(Pt 9):2266-77

Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA.

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http://dx.doi.org/10.1093/brain/awl224DOI Listing
September 2006

Gerstmann-Sträussler-Scheinker: a new phenotype with 'curly' PrP deposits.

J Neuropathol Exp Neurol 2006 Jul;65(7):642-51

Department of Neurology, University of Genoa, Genoa, Italy, and Pikeville Neurology Clinic and Diagnostic Center, Pikeville, Kentucky, USA.

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http://dx.doi.org/10.1097/01.jnen.0000228198.81797.4dDOI Listing
July 2006

Advances in Prion Disease Surveillance.

Adv Clin Chem 2006 6;41:263-292. Epub 2006 May 6.

National Prion Disease Pathology Surveillance Center, Institute of Pathology, Case Western Reserve University, Cleveland, Ohio.

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http://dx.doi.org/10.1016/S0065-2423(05)41008-2DOI Listing
May 2006

Redox metals and oxidative abnormalities in human prion diseases.

Acta Neuropathol 2005 Sep 11;110(3):232-8. Epub 2005 Aug 11.

Institute of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106, USA.

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http://dx.doi.org/10.1007/s00401-005-1034-4DOI Listing
September 2005

Variant Creutzfeldt-Jakob disease death, United States.

Emerg Infect Dis 2005 Sep;11(9):1351-4

Division of Viral and Rickettsial Disease, National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Georgia 30333, USA.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310634PMC
http://dx.doi.org/10.3201/eid1109.050371DOI Listing
September 2005

Tribute to Robert Terry.

Alzheimers Dement 2005 Jul;1(1):83

Department of Pathology, Case Western Reserve University, 2085 Adelbert Rd., Cleveland, OH, USA.

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http://dx.doi.org/10.1016/j.jalz.2005.06.020DOI Listing
July 2005

From microbes to prions the final proof of the prion hypothesis.

Cell 2005 Apr;121(2):155-7

Division of Neuropathology, Department of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH 44106, USA.

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http://dx.doi.org/10.1016/j.cell.2005.04.002DOI Listing
April 2005

Characterization of the F198S prion protein mutation: enhanced glycosylation and defective refolding.

J Alzheimers Dis 2005 Apr;7(2):159-71; discussion 173-80

Department of Physiology and Biophysics, Howard University College of Medicine, Washington, DC 20059, USA.

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April 2005

Concealment of epitope by reduction and alkylation in prion protein.

Biochem Biophys Res Commun 2005 Jan;326(3):652-9

Institute of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106, USA.

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http://dx.doi.org/10.1016/j.bbrc.2004.11.088DOI Listing
January 2005

Biochemical fingerprints of prion diseases: scrapie prion protein in human prion diseases that share prion genotype and type.

J Neurochem 2005 Jan;92(1):132-42

Division of Neuropathology, Case Western Reserve University, Cleveland, Ohio 44120, USA.

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http://dx.doi.org/10.1111/j.1471-4159.2004.02859.xDOI Listing
January 2005

Targeting prion amyloid deposits in vivo.

J Neuropathol Exp Neurol 2004 Jul;63(7):775-84

Department of Neurology, New York University School of Medicine, New York, New York 10016, USA.

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http://dx.doi.org/10.1093/jnen/63.7.775DOI Listing
July 2004

Chronic wasting disease and potential transmission to humans.

Emerg Infect Dis 2004 Jun;10(6):977-84

Centers for Disease Control and Prevention, Atlanta, Georgia 30333, USA.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3323184PMC
http://dx.doi.org/10.3201/eid1006.031082DOI Listing
June 2004

Antibody to DNA detects scrapie but not normal prion protein.

Proc Natl Acad Sci U S A 2004 Feb 20;101(5):1380-5. Epub 2004 Jan 20.

Institute of Pathology, Case Western Reserve University and National Prion Disease Pathology Surveillance Center, 2085 Adelbert Road, Cleveland, OH 44106, USA.

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http://dx.doi.org/10.1073/pnas.0307825100DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC337061PMC
February 2004

Soluble amyloid beta-protein is increased in frontotemporal dementia with tau gene mutations.

J Alzheimers Dis 2004 Feb;6(1):45-51

Department of Neurological Sciences, Psychiatry and Genetics, University of Genova, Italy.

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February 2004

Fatal familial insomnia: the first account in a family of Chinese descent.

Arch Neurol 2004 Jan;61(1):122-5

Division of Neurology, Department of Medicine, The University of British Columbia, S192-2211 Wesbrook Mall, Vancouver, BC, Canada V6L 1P8.

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http://archneur.jamanetwork.com/article.aspx?doi=10.1001/arc
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http://dx.doi.org/10.1001/archneur.61.1.122DOI Listing
January 2004

Sporadic and familial CJD: classification and characterisation.

Br Med Bull 2003 ;66:213-39

Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA.

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http://dx.doi.org/10.1093/bmb/66.1.213DOI Listing
November 2003

Identification of novel proteinase K-resistant C-terminal fragments of PrP in Creutzfeldt-Jakob disease.

J Biol Chem 2003 Oct 12;278(42):40429-36. Epub 2003 Aug 12.

Institute of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA.

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http://dx.doi.org/10.1074/jbc.M308550200DOI Listing
October 2003

Characterization of prion proteins.

Methods Mol Biol 2003 ;217:305-14

Institute of Pathology, Case Western Reserve University, National Prion Disease Pathology Surveillance Center, Cleveland, OH, USA.

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April 2003

Hereditary Creutzfeldt-Jakob disease and fatal familial insomnia.

Clin Lab Med 2003 Mar;23(1):43-64

Institute of Pathology, Case Western Reserve University, and National Prion Disease Pathology Surveillance Center, 2085 Adelbert Road, Cleveland, OH 44106-4907, USA.

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March 2003

Familial and sporadic fatal insomnia.

Lancet Neurol 2003 Mar;2(3):167-76

Department of Neurological Sciences, University of Bologna, Bologna, Italy.

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March 2003

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States.

Neurology 2003 Jan;60(2):176-81

Division of Viral and Rickettsial Diseases, National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, GA, USA.

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http://dx.doi.org/10.1212/01.wnl.0000036913.87823.d6DOI Listing
January 2003

Intercellular transfer of the cellular prion protein.

J Biol Chem 2002 Dec 30;277(49):47671-8. Epub 2002 Sep 30.

Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA.

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http://dx.doi.org/10.1074/jbc.M207458200DOI Listing
December 2002