Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene.

PubWeight™: 2.01‹?› | Rank: Top 2%

🔗 View Article (PMC 153633)

Published in Proc Natl Acad Sci U S A on April 08, 2003

Authors

Carsten Korth1, Kiyotoshi Kaneko, Darlene Groth, Norbert Heye, Glenn Telling, James Mastrianni, Piero Parchi, Pierluigi Gambetti, Robert Will, James Ironside, Cornelia Heinrich, Patrick Tremblay, Stephen J DeArmond, Stanley B Prusiner

Author Affiliations

1: Institute for Neurodegenerative Diseases and Department of Neurology, University of California, San Francisco, CA 94143, USA.

Articles citing this

Identification of a second bovine amyloidotic spongiform encephalopathy: molecular similarities with sporadic Creutzfeldt-Jakob disease. Proc Natl Acad Sci U S A (2004) 3.29

Transmission of prions from mule deer and elk with chronic wasting disease to transgenic mice expressing cervid PrP. J Virol (2004) 2.34

Diagnosis of human prion disease. Proc Natl Acad Sci U S A (2005) 2.13

Transmission of elk and deer prions to transgenic mice. J Virol (2006) 2.03

Conversion of the BASE prion strain into the BSE strain: the origin of BSE? PLoS Pathog (2007) 1.97

Accelerated high fidelity prion amplification within and across prion species barriers. PLoS Pathog (2008) 1.89

Immunodetection of disease-associated mutant PrP, which accelerates disease in GSS transgenic mice. EMBO J (2005) 1.83

Efficient transmission and characterization of Creutzfeldt-Jakob disease strains in bank voles. PLoS Pathog (2006) 1.70

Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein. Ann Neurol (2010) 1.66

Conformational transformation and selection of synthetic prion strains. J Mol Biol (2011) 1.66

Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties. Proc Natl Acad Sci U S A (2010) 1.51

Prion proteins in subpopulations of white blood cells from patients with sporadic Creutzfeldt-Jakob disease. Lab Invest (2009) 1.48

Observation of sequence specificity in the seeding of protein amyloid fibrils. Protein Sci (2004) 1.44

Transmission barriers for bovine, ovine, and human prions in transgenic mice. J Virol (2005) 1.43

Inactivation of prions by acidic sodium dodecyl sulfate. J Virol (2006) 1.43

Characterization of truncated forms of abnormal prion protein in Creutzfeldt-Jakob disease. J Biol Chem (2008) 1.40

Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification. Acta Neuropathol (2009) 1.32

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

Prominent and persistent extraneural infection in human PrP transgenic mice infected with variant CJD. PLoS One (2008) 1.24

Runaway domain swapping in amyloid-like fibrils of T7 endonuclease I. Proc Natl Acad Sci U S A (2006) 1.16

Humanized knock-in mice expressing chimeric prion protein showed varied susceptibility to different human prions. Am J Pathol (2003) 1.16

Prion strain-dependent differences in conversion of mutant prion proteins in cell culture. J Virol (2006) 1.14

Resistance of bovine spongiform encephalopathy (BSE) prions to inactivation. PLoS Pathog (2008) 1.14

Antihypertensive drug guanabenz is active in vivo against both yeast and mammalian prions. PLoS One (2008) 1.13

Agent strain variation in human prion disease: insights from a molecular and pathological review of the National Institutes of Health series of experimentally transmitted disease. Brain (2010) 1.12

Review: contribution of transgenic models to understanding human prion disease. Neuropathol Appl Neurobiol (2010) 1.11

Small protease sensitive oligomers of PrPSc in distinct human prions determine conversion rate of PrP(C). PLoS Pathog (2012) 1.07

Human prion strain selection in transgenic mice. Ann Neurol (2010) 1.06

PRNP variation in UK sporadic and variant Creutzfeldt Jakob disease highlights genetic risk factors and a novel non-synonymous polymorphism. BMC Med Genet (2009) 1.06

Molecular biology and pathology of prion strains in sporadic human prion diseases. Acta Neuropathol (2010) 1.06

Evidence that bank vole PrP is a universal acceptor for prions. PLoS Pathog (2014) 1.03

Strain-specific viral properties of variant Creutzfeldt-Jakob disease (vCJD) are encoded by the agent and not by host prion protein. J Cell Biochem (2009) 1.01

Molecular pathology of human prion disease. Acta Neuropathol (2010) 1.01

Small ruminant nor98 prions share biochemical features with human gerstmann-sträussler-scheinker disease and variably protease-sensitive prionopathy. PLoS One (2013) 1.00

Mouse models for studying the formation and propagation of prions. J Biol Chem (2014) 0.98

Experimental verification of a traceback phenomenon in prion infection. J Virol (2010) 0.96

Iatrogenic CJD due to pituitary-derived growth hormone with genetically determined incubation times of up to 40 years. Brain (2015) 0.95

Rapid and Quantitative Assay of Amyloid-Seeding Activity in Human Brains Affected with Prion Diseases. PLoS One (2015) 0.93

Pharmacokinetics and metabolism of 2-aminothiazoles with antiprion activity in mice. Pharm Res (2013) 0.92

Identification of I137M and other mutations that modulate incubation periods for two human prion strains. J Virol (2012) 0.86

Chimeric elk/mouse prion proteins in transgenic mice. J Gen Virol (2012) 0.85

Co-existence of distinct prion types enables conformational evolution of human PrPSc by competitive selection. J Biol Chem (2013) 0.83

Modulation of Creutzfeldt-Jakob disease prion propagation by the A224V mutation. Ann Neurol (2015) 0.82

Phosphorothioate oligonucleotides reduce PrP levels and prion infectivity in cultured cells. Mol Med (2007) 0.82

Prion protein misfolding, strains, and neurotoxicity: an update from studies on Mammalian prions. Int J Cell Biol (2013) 0.82

No major change in vCJD agent strain after secondary transmission via blood transfusion. PLoS One (2008) 0.81

The glycosylation status of PrPC is a key factor in determining transmissible spongiform encephalopathy transmission between species. J Virol (2015) 0.81

Live cell fluorescence resonance energy transfer predicts an altered molecular association of heterologous PrPSc with PrPC. J Biol Chem (2010) 0.81

Transmission Properties of Human PrP 102L Prions Challenge the Relevance of Mouse Models of GSS. PLoS Pathog (2015) 0.78

Structural effects of PrP polymorphisms on intra- and interspecies prion transmission. Proc Natl Acad Sci U S A (2014) 0.78

Nucleic acid-free mutation of prion strains. Prion (2010) 0.78

Conserved properties of human and bovine prion strains on transmission to guinea pigs. Lab Invest (2011) 0.78

Identifying critical sites of PrP(c)-PrP(Sc) interaction in prion-infected cells by dominant-negative inhibition. Prion (2013) 0.77

The mechanism of prion strain propagation. Genome Biol (2004) 0.76

Insights into prion biology: integrating a protein misfolding pathway with its cellular environment. Prion (2011) 0.75

Guinea Pig Prion Protein Supports Rapid Propagation of Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease Prions. J Virol (2016) 0.75

Assessing transmissible spongiform encephalopathy species barriers with an in vitro prion protein conversion assay. J Vis Exp (2015) 0.75

Bioassays and Inactivation of Prions. Cold Spring Harb Perspect Biol (2017) 0.75

Articles cited by this

Prions. Proc Natl Acad Sci U S A (1998) 27.80

A new variant of Creutzfeldt-Jakob disease in the UK. Lancet (1996) 20.15

Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature (1997) 15.73

Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature (1996) 14.40

Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein. Nature (1992) 9.01

Eight prion strains have PrP(Sc) molecules with different conformations. Nat Med (1998) 9.01

Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol (1999) 6.98

Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity. Science (1996) 6.71

Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication. Cell (1990) 6.17

Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein. Cell (1995) 5.16

Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy. J Virol (1994) 5.12

Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease. Nature (1991) 5.04

Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques. Cell (1989) 4.73

Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation. Proc Natl Acad Sci U S A (1997) 3.78

Regional mapping of prion proteins in brain. Proc Natl Acad Sci U S A (1992) 3.68

Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions. Cell (1998) 3.59

Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proc Natl Acad Sci U S A (1999) 3.43

A change in the conformation of prions accompanies the emergence of a new prion strain. Neuron (2002) 3.36

BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J (2002) 2.98

Replication of distinct scrapie prion isolates is region specific in brains of transgenic mice and hamsters. Genes Dev (1992) 2.87

Transmissible and genetic prion diseases share a common pathway of neurodegeneration. Nature (1999) 2.83

Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein. Proc Natl Acad Sci U S A (1994) 2.70

Chimeric prion protein expression in cultured cells and transgenic mice. Protein Sci (1992) 2.67

Precise targeting of the pathology of the sialoglycoprotein, PrP, and vacuolar degeneration in mouse scrapie. Neurosci Lett (1989) 2.48

Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice. Genes Dev (1996) 2.39

Prion isolate specified allotypic interactions between the cellular and scrapie prion proteins in congenic and transgenic mice. Proc Natl Acad Sci U S A (1994) 2.38

Temporary and permanent modifications to a single strain of mouse scrapie on transmission to rats and hamsters. J Gen Virol (1987) 2.27

Measuring prions causing bovine spongiform encephalopathy or chronic wasting disease by immunoassays and transgenic mice. Nat Biotechnol (2002) 2.24

Scrapie prions. Annu Rev Microbiol (1989) 2.14

Prion protein conformation in a patient with sporadic fatal insomnia. N Engl J Med (1999) 2.04

Heterologous PrP molecules interfere with accumulation of protease-resistant PrP in scrapie-infected murine neuroblastoma cells. J Virol (1994) 1.85

Onset of ataxia and Purkinje cell loss in PrP null mice inversely correlated with Dpl level in brain. EMBO J (2001) 1.85

Heritable disorder resembling neuronal storage disease in mice expressing prion protein with deletion of an alpha-helix. Nat Med (1997) 1.83

A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination. Lab Invest (1999) 1.83

Selective neuronal targeting in prion disease. Neuron (1997) 1.82

Dominant-negative inhibition of prion replication in transgenic mice. Proc Natl Acad Sci U S A (2002) 1.65

Identification of two prion protein regions that modify scrapie incubation time. J Virol (2001) 1.63

Doppel-induced cerebellar degeneration in transgenic mice. Proc Natl Acad Sci U S A (2001) 1.52

A comparison of some biological characteristics of the mouse-passaged scrapie agents, 22A and ME7. Genet Res (1969) 1.45

Nucleic acids in prion preparations: unspecific background or essential component? Philos Trans R Soc Lond B Biol Sci (1994) 1.01

A protease-resistant 61-residue prion peptide causes neurodegeneration in transgenic mice. Mol Cell Biol (2001) 0.98

Successful transmission of Creutzfeldt-Jakob disease from human to mouse verified by prion protein accumulation in mouse brains. Brain Res (1992) 0.90

Articles by these authors

Synthetic mammalian prions. Science (2004) 7.35

Sporadic and familial CJD: classification and characterisation. Br Med Bull (2003) 3.66

Evidence for assembly of prions with left-handed beta-helices into trimers. Proc Natl Acad Sci U S A (2004) 3.64

Primary postoperative chemotherapy without radiotherapy for intracranial ependymoma in children: the UKCCSG/SIOP prospective study. Lancet Oncol (2007) 3.47

A prion protein epitope selective for the pathologically misfolded conformation. Nat Med (2003) 3.41

A change in the conformation of prions accompanies the emergence of a new prion strain. Neuron (2002) 3.36

Challenging the clinical utility of the 14-3-3 protein for the diagnosis of sporadic Creutzfeldt-Jakob disease. Arch Neurol (2003) 3.20

Prions. Cold Spring Harb Perspect Biol (2011) 3.17

Small-molecule aggregates inhibit amyloid polymerization. Nat Chem Biol (2008) 2.98

Pathway complexity of prion protein assembly into amyloid. J Biol Chem (2002) 2.96

Structural studies of the scrapie prion protein by electron crystallography. Proc Natl Acad Sci U S A (2002) 2.91

Continuum of prion protein structures enciphers a multitude of prion isolate-specified phenotypes. Proc Natl Acad Sci U S A (2006) 2.69

Genetic prion disease: the EUROCJD experience. Hum Genet (2005) 2.64

Mammalian prions generated from bacterially expressed prion protein in the absence of any mammalian cofactors. J Biol Chem (2010) 2.57

Chronic wasting disease of elk: transmissibility to humans examined by transgenic mouse models. J Neurosci (2005) 2.46

Design and construction of diverse mammalian prion strains. Proc Natl Acad Sci U S A (2009) 2.41

Analysis of proteome dynamics in the mouse brain. Proc Natl Acad Sci U S A (2010) 2.37

Purified and synthetic Alzheimer's amyloid beta (Aβ) prions. Proc Natl Acad Sci U S A (2012) 2.36

Prion detection by an amyloid seeding assay. Proc Natl Acad Sci U S A (2007) 2.34

Strain-specified characteristics of mouse synthetic prions. Proc Natl Acad Sci U S A (2005) 2.25

Measuring prions causing bovine spongiform encephalopathy or chronic wasting disease by immunoassays and transgenic mice. Nat Biotechnol (2002) 2.24

Transmission and detection of prions in feces. J Infect Dis (2008) 2.20

Cell-free propagation of prion strains. EMBO J (2008) 2.20

Transmission of multiple system atrophy prions to transgenic mice. Proc Natl Acad Sci U S A (2013) 2.19

Protease-sensitive synthetic prions. PLoS Pathog (2010) 2.14

Diagnosis of human prion disease. Proc Natl Acad Sci U S A (2005) 2.13

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

Asymptomatic deer excrete infectious prions in faeces. Nature (2009) 2.10

Mutant PrPSc conformers induced by a synthetic peptide and several prion strains. J Virol (2004) 2.07

The clinical presentation of mitochondrial diseases in children with progressive intellectual and neurological deterioration: a national, prospective, population-based study. Dev Med Child Neurol (2009) 2.05

Transmission of elk and deer prions to transgenic mice. J Virol (2006) 2.03

Natural and synthetic prion structure from X-ray fiber diffraction. Proc Natl Acad Sci U S A (2009) 1.97

Mouse syngenic in vitro blood-brain barrier model: a new tool to examine inflammatory events in cerebral endothelium. Lab Invest (2005) 1.92

CSF biomarker variability in the Alzheimer's Association quality control program. Alzheimers Dement (2013) 1.90

Results of a randomized study of preradiation chemotherapy versus radiotherapy alone for nonmetastatic medulloblastoma: The International Society of Paediatric Oncology/United Kingdom Children's Cancer Study Group PNET-3 Study. J Clin Oncol (2003) 1.87

Chronic wasting disease and potential transmission to humans. Emerg Infect Dis (2004) 1.87

Deficiency in neuronal TGF-beta signaling promotes neurodegeneration and Alzheimer's pathology. J Clin Invest (2006) 1.87

Clinicopathological correlations in corticobasal degeneration. Ann Neurol (2011) 1.86

Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease. Ann Neurol (2011) 1.86

Staging of neurofibrillary pathology in Alzheimer's disease: a study of the BrainNet Europe Consortium. Brain Pathol (2008) 1.85

Prion clearance in bigenic mice. J Gen Virol (2005) 1.79

Pre-symptomatic detection of prions by cyclic amplification of protein misfolding. FEBS Lett (2005) 1.77

Sporadic human prion diseases: molecular insights and diagnosis. Lancet Neurol (2012) 1.75

Prions in the urine of patients with variant Creutzfeldt-Jakob disease. N Engl J Med (2014) 1.74

Diffusion-weighted brain imaging study of patients with clinical diagnosis of corticobasal degeneration, progressive supranuclear palsy and Parkinson's disease. Brain (2008) 1.69

Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein. Ann Neurol (2010) 1.66

Conformational transformation and selection of synthetic prion strains. J Mol Biol (2011) 1.66

Copper coordination in the full-length, recombinant prion protein. Biochemistry (2003) 1.66

Dominant-negative inhibition of prion replication in transgenic mice. Proc Natl Acad Sci U S A (2002) 1.65

Familial and sporadic fatal insomnia. Lancet Neurol (2003) 1.65

Time-controlled transcardiac perfusion cross-linking for the study of protein interactions in complex tissues. Nat Biotechnol (2004) 1.63

PrP conformational transitions alter species preference of a PrP-specific antibody. J Biol Chem (2010) 1.62

Identification of novel proteinase K-resistant C-terminal fragments of PrP in Creutzfeldt-Jakob disease. J Biol Chem (2003) 1.61

Measuring prions by bioluminescence imaging. Proc Natl Acad Sci U S A (2009) 1.59

Evaluation of the human transmission risk of an atypical bovine spongiform encephalopathy prion strain. J Virol (2008) 1.59

Continuous quinacrine treatment results in the formation of drug-resistant prions. PLoS Pathog (2009) 1.58

Insoluble aggregates and protease-resistant conformers of prion protein in uninfected human brains. J Biol Chem (2006) 1.57

Is Parkinson's disease a prion disorder? Proc Natl Acad Sci U S A (2009) 1.56

Interlaboratory comparison of assessments of Alzheimer disease-related lesions: a study of the BrainNet Europe Consortium. J Neuropathol Exp Neurol (2006) 1.56

Intercellular transfer of the cellular prion protein. J Biol Chem (2002) 1.54

Polio and Nobel prizes: looking back 50 years. Ann Neurol (2007) 1.54

Classification of sporadic Creutzfeldt-Jakob disease revisited. Brain (2006) 1.54

Genes contributing to prion pathogenesis. J Gen Virol (2008) 1.51

Management of a twenty-first century brain bank: experience in the BrainNet Europe consortium. Acta Neuropathol (2008) 1.51

Trafficking of prion proteins through a caveolae-mediated endosomal pathway. J Cell Biol (2003) 1.51

Discovery of 2-aminothiazoles as potent antiprion compounds. J Virol (2009) 1.51

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