PubRank

Pierluigi Gambetti

Author PubWeight™ 79.17‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Mammalian prions generated from bacterially expressed prion protein in the absence of any mammalian cofactors. J Biol Chem 2010 2.57
2 Chronic wasting disease of elk: transmissibility to humans examined by transgenic mouse models. J Neurosci 2005 2.46
3 Cell-free propagation of prion strains. EMBO J 2008 2.20
4 Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics. Brain 2009 2.12
5 Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene. Proc Natl Acad Sci U S A 2003 2.01
6 Chronic wasting disease and potential transmission to humans. Emerg Infect Dis 2004 1.87
7 Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease. Ann Neurol 2011 1.86
8 Sporadic human prion diseases: molecular insights and diagnosis. Lancet Neurol 2012 1.75
9 Prions in the urine of patients with variant Creutzfeldt-Jakob disease. N Engl J Med 2014 1.74
10 Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein. Ann Neurol 2010 1.66
11 Familial and sporadic fatal insomnia. Lancet Neurol 2003 1.65
12 Identification of novel proteinase K-resistant C-terminal fragments of PrP in Creutzfeldt-Jakob disease. J Biol Chem 2003 1.61
13 Evaluation of the human transmission risk of an atypical bovine spongiform encephalopathy prion strain. J Virol 2008 1.59
14 Insoluble aggregates and protease-resistant conformers of prion protein in uninfected human brains. J Biol Chem 2006 1.57
15 Intercellular transfer of the cellular prion protein. J Biol Chem 2002 1.54
16 Classification of sporadic Creutzfeldt-Jakob disease revisited. Brain 2006 1.54
17 Effects of different experimental conditions on the PrPSc core generated by protease digestion: implications for strain typing and molecular classification of CJD. J Biol Chem 2004 1.50
18 beta-amyloid is different in normal aging and in Alzheimer disease. J Biol Chem 2005 1.48
19 Antibody to DNA detects scrapie but not normal prion protein. Proc Natl Acad Sci U S A 2004 1.43
20 Characterization of truncated forms of abnormal prion protein in Creutzfeldt-Jakob disease. J Biol Chem 2008 1.40
21 Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in Europe and USA. Acta Neuropathol 2012 1.29
22 Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States. Neurology 2003 1.19
23 Heterogeneity of normal prion protein in two- dimensional immunoblot: presence of various glycosylated and truncated forms. J Neurochem 2002 1.18
24 Pre-symptomatic diagnosis in fatal familial insomnia: serial neurophysiological and 18FDG-PET studies. Brain 2006 1.18
25 Protease-resistant human prion protein and ferritin are cotransported across Caco-2 epithelial cells: implications for species barrier in prion uptake from the intestine. J Neurosci 2004 1.17
26 Fatal familial insomnia: the first account in a family of Chinese descent. Arch Neurol 2004 1.13
27 Generation of a new form of human PrP(Sc) in vitro by interspecies transmission from cervid prions. J Biol Chem 2011 1.12
28 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 1.12
29 Spontaneous mutations in the prion protein gene causing transmissible spongiform encephalopathy. Ann Neurol 2002 1.07
30 The role of glycophosphatidylinositol anchor in the amplification of the scrapie isoform of prion protein in vitro. FEBS Lett 2009 1.04
31 Multiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United States. PLoS One 2010 1.00
32 Small ruminant nor98 prions share biochemical features with human gerstmann-sträussler-scheinker disease and variably protease-sensitive prionopathy. PLoS One 2013 1.00
33 Redox metals and oxidative abnormalities in human prion diseases. Acta Neuropathol 2005 0.98
34 Chronic wasting disease of elk and deer and Creutzfeldt-Jakob disease: comparative analysis of the scrapie prion protein. J Biol Chem 2005 0.96
35 Inducible overexpression of wild-type prion protein in the muscles leads to a primary myopathy in transgenic mice. Proc Natl Acad Sci U S A 2007 0.94
36 Creutzfeldt-Jakob disease (CJD) with a mutation at codon 148 of prion protein gene: relationship with sporadic CJD. Am J Pathol 2005 0.93
37 Gerstmann-Sträussler-Scheinker: a new phenotype with 'curly' PrP deposits. J Neuropathol Exp Neurol 2006 0.92
38 Codistribution of amyloid beta plaques and spongiform degeneration in familial Creutzfeldt-Jakob disease with the E200K-129M haplotype. Arch Neurol 2009 0.91
39 Glycoform-selective prion formation in sporadic and familial forms of prion disease. PLoS One 2013 0.91
40 Targeting prion amyloid deposits in vivo. J Neuropathol Exp Neurol 2004 0.91
41 Biochemical fingerprints of prion diseases: scrapie prion protein in human prion diseases that share prion genotype and type. J Neurochem 2005 0.90
42 A journey through the species barrier. Neuron 2002 0.90
43 Concealment of epitope by reduction and alkylation in prion protein. Biochem Biophys Res Commun 2005 0.89
44 Does the prion protein gene 129 codon polymorphism influence sleep? Evidence from a fatal familial insomnia kindred. Clin Neurophysiol 2002 0.87
45 Characterization of the prion protein in human urine. J Biol Chem 2010 0.86
46 Recent US Case of Variant Creutzfeldt-Jakob Disease-Global Implications. Emerg Infect Dis 2015 0.86
47 From microbes to prions the final proof of the prion hypothesis. Cell 2005 0.86
48 Failure to detect the presence of prions in the uterine and gestational tissues from a Gravida with Creutzfeldt-Jakob disease. Am J Pathol 2009 0.85
49 Characterization of the F198S prion protein mutation: enhanced glycosylation and defective refolding. J Alzheimers Dis 2005 0.84
50 Protease-sensitive prions with 144-bp insertion mutations. Aging (Albany NY) 2013 0.84
51 Soluble amyloid beta-protein is increased in frontotemporal dementia with tau gene mutations. J Alzheimers Dis 2004 0.83
52 Constant transmission properties of variant Creutzfeldt-Jakob disease in 5 countries. Emerg Infect Dis 2012 0.81
53 Characterization of prion proteins. Methods Mol Biol 2003 0.81
54 Soluble amyloid-beta in the brain: the scarlet pimpernel. J Alzheimers Dis 2006 0.80
55 Human prion diseases. Med Clin North Am 2002 0.80
56 Expression of excitatory amino acid transporter-1 (EAAT-1) in brain macrophages and microglia of patients with prion diseases. J Neuropathol Exp Neurol 2004 0.80
57 Variant Creutzfeldt-Jakob disease death, United States. Emerg Infect Dis 2005 0.79
58 Correction: Bank Vole Prion Protein As an Apparently Universal Substrate for RT-QuIC-Based Detection and Discrimination of Prion Strains. PLoS Pathog 2015 0.79
59 Washington statewide pathology surveillance for prion disease. Ann Neurol 2007 0.79
60 Transmission characteristics of variably protease-sensitive prionopathy. Emerg Infect Dis 2014 0.79
61 Sporadic fatal insomnia masquerading as a paraneoplastic cerebellar syndrome. Arch Neurol 2008 0.79
62 Toxic effects of intracerebral PrP antibody administration during the course of BSE infection in mice. Prion 2007 0.78
63 Thermodynamic stabilization of the folded domain of prion protein inhibits prion infection in vivo. Cell Rep 2013 0.77
64 Assessing prion infectivity of human urine in sporadic Creutzfeldt-Jakob disease. Emerg Infect Dis 2012 0.77
65 Genetic CJD with a novel E200G mutation in the prion protein gene and comparison with E200K mutation cases. Acta Neuropathol Commun 2013 0.77
66 Variant Creutzfeldt-Jakob disease: French versus British. Ann Neurol 2009 0.75
67 Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) Correlation of Histopathology and MRI in Prion Disease. Alzheimer Dis Assoc Disord 2017 0.75
68 Variably Protease-sensitive Prionopathy in an Apparent Cognitively Normal 93-Year-Old. Alzheimer Dis Assoc Disord 2015 0.75
69 Sporadic Creutzfeldt-Jakob disease in a native Puerto Rican patient. P R Health Sci J 2015 0.75