Pierluigi Gambetti

Summary

Affiliation: Case Western Reserve University
Country: USA

Publications

  1. pmc Creationism and evolutionism in prions
    Pierluigi Gambetti
    Department of Pathology, Case Western Reserve University, Cleveland, Ohio Electronic address
    Am J Pathol 182:623-7. 2013
  2. pmc Molecular biology and pathology of prion strains in sporadic human prion diseases
    Pierluigi Gambetti
    Department of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH, 44106, USA
    Acta Neuropathol 121:79-90. 2011
  3. pmc Evaluation of the human transmission risk of an atypical bovine spongiform encephalopathy prion strain
    Qingzhong Kong
    Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
    J Virol 82:3697-701. 2008
  4. pmc A novel human disease with abnormal prion protein sensitive to protease
    Pierluigi Gambetti
    Institute of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
    Ann Neurol 63:697-708. 2008
  5. pmc Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics
    Ignazio Cali
    Department of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106, USA
    Brain 132:2643-58. 2009
  6. ncbi request reprint Insoluble aggregates and protease-resistant conformers of prion protein in uninfected human brains
    Jue Yuan
    Department of Pathology and National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 281:34848-58. 2006
  7. pmc Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein
    Wen Quan Zou
    Department of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH 44106, USA
    Ann Neurol 68:162-72. 2010
  8. ncbi request reprint Sporadic and familial CJD: classification and characterisation
    Pierluigi Gambetti
    Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA
    Br Med Bull 66:213-39. 2003
  9. ncbi request reprint Classification of sporadic Creutzfeldt-Jakob disease revisited
    Ignazio Cali
    Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
    Brain 129:2266-77. 2006
  10. pmc Creutzfeldt-Jakob disease (CJD) with a mutation at codon 148 of prion protein gene: relationship with sporadic CJD
    Manuela Pastore
    Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
    Am J Pathol 167:1729-38. 2005

Collaborators

Detail Information

Publications37

  1. pmc Creationism and evolutionism in prions
    Pierluigi Gambetti
    Department of Pathology, Case Western Reserve University, Cleveland, Ohio Electronic address
    Am J Pathol 182:623-7. 2013
    ....
  2. pmc Molecular biology and pathology of prion strains in sporadic human prion diseases
    Pierluigi Gambetti
    Department of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH, 44106, USA
    Acta Neuropathol 121:79-90. 2011
    ..However, the intrinsic characteristics that distinguish at least four of these strains remain to be identified...
  3. pmc Evaluation of the human transmission risk of an atypical bovine spongiform encephalopathy prion strain
    Qingzhong Kong
    Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
    J Virol 82:3697-701. 2008
    ..Minimal brain spongiosis and long incubation times are observed for the BASE strain-infected Tg mice. These results suggest that in humans, the BASE strain is a more virulent BSE strain and likely lymphotropic...
  4. pmc A novel human disease with abnormal prion protein sensitive to protease
    Pierluigi Gambetti
    Institute of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
    Ann Neurol 63:697-708. 2008
    ....
  5. pmc Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics
    Ignazio Cali
    Department of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106, USA
    Brain 132:2643-58. 2009
    ..All of these findings indicate that sCJDMM1-2 should be considered as a separate entity at this time...
  6. ncbi request reprint Insoluble aggregates and protease-resistant conformers of prion protein in uninfected human brains
    Jue Yuan
    Department of Pathology and National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 281:34848-58. 2006
    ..This is the first study that provides experimental evidence supporting the hypothesis that there might be silent prions lying dormant in normal human brains...
  7. pmc Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein
    Wen Quan Zou
    Department of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH 44106, USA
    Ann Neurol 68:162-72. 2010
    ..The 2 new PSPr forms affect individuals who are either homozygous for methionine (129MM) or heterozygous for methionine/valine (129MV)...
  8. ncbi request reprint Sporadic and familial CJD: classification and characterisation
    Pierluigi Gambetti
    Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA
    Br Med Bull 66:213-39. 2003
    ..The clinical and pathological features are summarised for each sporadic CJD subtype and familial CJD haplotype...
  9. ncbi request reprint Classification of sporadic Creutzfeldt-Jakob disease revisited
    Ignazio Cali
    Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
    Brain 129:2266-77. 2006
    ..These data indicate that the differentiation of sCJDMM1 into two subgroups is not currently justified...
  10. pmc Creutzfeldt-Jakob disease (CJD) with a mutation at codon 148 of prion protein gene: relationship with sporadic CJD
    Manuela Pastore
    Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
    Am J Pathol 167:1729-38. 2005
    ....
  11. pmc Mammalian prions generated from bacterially expressed prion protein in the absence of any mammalian cofactors
    Jae Il Kim
    Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 285:14083-7. 2010
    ....
  12. ncbi request reprint Protease-resistant human prion protein and ferritin are cotransported across Caco-2 epithelial cells: implications for species barrier in prion uptake from the intestine
    Ravi Shankar Mishra
    Institute of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Neurosci 24:11280-90. 2004
    ....
  13. ncbi request reprint Chronic wasting disease of elk and deer and Creutzfeldt-Jakob disease: comparative analysis of the scrapie prion protein
    Zhiliang Xie
    Institute of Pathology and National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 281:4199-206. 2006
    ..These findings underline the importance of detailed PrP(Sc) characterization in trying to detect novel forms of acquired prion disease...
  14. pmc Failure to detect the presence of prions in the uterine and gestational tissues from a Gravida with Creutzfeldt-Jakob disease
    Xiangzhu Xiao
    Department of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA
    Am J Pathol 174:1602-8. 2009
    ..Nevertheless, examination by a highly sensitive bioassay is ongoing to ascertain possible prion infectivity from CJD in the amniotic fluid...
  15. pmc Multiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United States
    Silvio Notari
    Institute of Pathology, Case Western Reserve University, Cleveland, Ohio, United States of America
    PLoS ONE 5:e8765. 2010
    ..Three cases have been identified in the United States; however, these subjects were likely exposed to prion infection elsewhere. Here we report on the first of these subjects...
  16. pmc Inducible overexpression of wild-type prion protein in the muscles leads to a primary myopathy in transgenic mice
    Shenghai Huang
    Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
    Proc Natl Acad Sci U S A 104:6800-5. 2007
    ....
  17. ncbi request reprint Identification of novel proteinase K-resistant C-terminal fragments of PrP in Creutzfeldt-Jakob disease
    Wen Quan Zou
    Institute of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 278:40429-36. 2003
    ..The finding of PrP-CTF12/13 in CJD brains widens the heterogeneity of the PK-resistant PrP fragments associated with prion diseases and may provide useful insights toward the understanding of the PrPSc structure and its formation...
  18. pmc Protease-sensitive prions with 144-bp insertion mutations
    Xiangzhu Xiao
    Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
    Aging (Albany NY) 5:155-73. 2013
    ....
  19. ncbi request reprint Characterization of prion proteins
    Wenquan Zou
    Institute of Pathology, Case Western Reserve University, National Prion Disease Pathology Surveillance Center, Cleveland, OH, USA
    Methods Mol Biol 217:305-14. 2003
  20. pmc The role of glycophosphatidylinositol anchor in the amplification of the scrapie isoform of prion protein in vitro
    Jae Il Kim
    Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, OH 44106, USA
    FEBS Lett 583:3671-5. 2009
    ..These findings shed a new light on prion protein conversion process and have important implications for the efforts to generate synthetic prions for structural and biophysical studies...
  21. ncbi request reprint Biochemical fingerprints of prion diseases: scrapie prion protein in human prion diseases that share prion genotype and type
    Tao Pan
    Division of Neuropathology, Case Western Reserve University, Cleveland, Ohio 44120, USA
    J Neurochem 92:132-42. 2005
    ..Overall, these findings underline the complexity of phenotypic determination in human prion diseases...
  22. pmc Thermodynamic Stabilization of the Folded Domain of Prion Protein Inhibits Prion Infection in Vivo
    Qingzhong Kong
    Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA Electronic address
    Cell Rep 4:248-54. 2013
    ....
  23. pmc Antibody to DNA detects scrapie but not normal prion protein
    Wen Quan Zou
    Institute of Pathology, Case Western Reserve University and National Prion Disease Pathology Surveillance Center, 2085 Adelbert Road, Cleveland, OH 44106, USA
    Proc Natl Acad Sci U S A 101:1380-5. 2004
    ..Our finding that anti-DNA Abs and gene 5 protein specifically target disease-associated DNA-PrP complexes in a wide variety of species and disease phenotypes opens new avenues in the study and diagnosis of prion diseases...
  24. ncbi request reprint Redox metals and oxidative abnormalities in human prion diseases
    Robert B Petersen
    Institute of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106, USA
    Acta Neuropathol 110:232-8. 2005
    ..These findings suggest an important distinction in prion-related oxidative stress, indicating that different neurodegenerative pathways are involved in different prion diseases...
  25. ncbi request reprint Chronic wasting disease of elk: transmissibility to humans examined by transgenic mouse models
    Qingzhong Kong
    Department of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Neurosci 25:7944-9. 2005
    ..These data indicate that there is a substantial species barrier for transmission of elk CWD to humans...
  26. ncbi request reprint Intercellular transfer of the cellular prion protein
    Tong Liu
    Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 277:47671-8. 2002
    ..These findings suggest that the intercellular transfer of GPI-anchored proteins is a regulated process, and may have implications for the pathogenesis of prion disease...
  27. ncbi request reprint Concealment of epitope by reduction and alkylation in prion protein
    Jue Yuan
    Institute of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106, USA
    Biochem Biophys Res Commun 326:652-9. 2005
    ..The implications in the conversion of PrP(C) into PrP(Sc) and the therapeutics of prion diseases are discussed...
  28. pmc Characterization of the prion protein in human urine
    Ayuna Dagdanova
    Department of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 285:30489-95. 2010
    ..The detailed characterization of uPrP reported here definitely proves the presence of PrP in human urine and will help determine the origin of prion infectivity in urine...
  29. doi request reprint Variably protease-sensitive prionopathy: a novel disease of the prion protein
    Pierluigi Gambetti
    Department of Pathology, National Prion Disease Pathology Surveillance Center, School of Medicine, Case Western Reserve University, Cleveland, OH 44106, USA
    J Mol Neurosci 45:422-4. 2011
    ..These findings suggest that VPSPr is basically different from classical prion diseases such as sCJD being perhaps more akin to other neurodegenerative dementias...
  30. pmc Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease
    Numthip Chitravas
    Department of Neurology, University Hospitals Case Medical Center, Cleveland, OH 44106, USA
    Ann Neurol 70:437-44. 2011
    ..Our goal was to determine which treatable disorders are most commonly mistaken for CJD...
  31. ncbi request reprint Human prion diseases
    Man Sun Sy
    Division of Neuropathology, Institute of Pathology, and Cancer Research Center, Room 933 Biomedical Research Building, School of Medicine, Case Western Reserve University, 10900 Euclid Avenue, Cleveland, OH 44120 1712, USA
    Med Clin North Am 86:551-71, vi-vii. 2002
    ..This article describes these diseases, current treatment modalities, and suggests directions for future research...
  32. pmc Assessing prion infectivity of human urine in sporadic Creutzfeldt-Jakob disease
    Silvio Notari
    Case Western Reserve University, Cleveland, Ohio, USA
    Emerg Infect Dis 18:21-8. 2012
    ..38 infectious units/mL...
  33. ncbi request reprint Hereditary Creutzfeldt-Jakob disease and fatal familial insomnia
    Pierluigi Gambetti
    Institute of Pathology, Case Western Reserve University, and National Prion Disease Pathology Surveillance Center, 2085 Adelbert Road, Cleveland, OH 44106 4907, USA
    Clin Lab Med 23:43-64. 2003
    ..This knowledge will enable the development of a rational and effective strategy for therapeutic intervention...
  34. ncbi request reprint From microbes to prions the final proof of the prion hypothesis
    Wen Quan Zou
    Division of Neuropathology, Department of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH 44106, USA
    Cell 121:155-7. 2005
    ..In this issue of Cell, come very close to accomplishing this goal by producing a prion disease in a natural host from a prion entirely generated in vitro using a PCR-like amplification system...
  35. pmc Glycoform-selective prion formation in sporadic and familial forms of prion disease
    Xiangzhu Xiao
    Department of Pathology, Case Western Reserve University School of Medicine, Cleveland, Ohio, United States of America
    PLoS ONE 8:e58786. 2013
    ....
  36. ncbi request reprint Washington statewide pathology surveillance for prion disease
    Camilla T Allen
    Department of Pathology, University of Washington, Seattle, WA 98104, USA
    Ann Neurol 61:371-2. 2007
    ..Our results do not support the hypotheses that variant CJD is an emerging illness in Washington or that sporadic CJD is more common in this state than in other regions of the world...
  37. ncbi request reprint A journey through the species barrier
    Shu G Chen
    Institute of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA
    Neuron 34:854-6. 2002
    ....