Published in N Engl J Med on May 17, 2001
Understanding protein non-folding. Biochim Biophys Acta (2010) 4.33
Evidence for assembly of prions with left-handed beta-helices into trimers. Proc Natl Acad Sci U S A (2004) 3.64
Acridine and phenothiazine derivatives as pharmacotherapeutics for prion disease. Proc Natl Acad Sci U S A (2001) 3.39
Prions. Cold Spring Harb Perspect Biol (2011) 3.17
Pathogenic protein seeding in Alzheimer disease and other neurodegenerative disorders. Ann Neurol (2011) 2.94
Huntington toxicity in yeast model depends on polyglutamine aggregation mediated by a prion-like protein Rnq1. J Cell Biol (2002) 2.76
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
Seeded aggregation and toxicity of {alpha}-synuclein and tau: cellular models of neurodegenerative diseases. J Biol Chem (2010) 1.92
A decade and a half of protein intrinsic disorder: biology still waits for physics. Protein Sci (2013) 1.74
Species-dependent differences in cofactor utilization for formation of the protease-resistant prion protein in vitro. Biochemistry (2010) 1.68
Conformational transformation and selection of synthetic prion strains. J Mol Biol (2011) 1.66
Protein aggregation in disease: a role for folding intermediates forming specific multimeric interactions. J Clin Invest (2002) 1.65
The function of α-synuclein. Neuron (2013) 1.63
Biology and genetics of prions causing neurodegeneration. Annu Rev Genet (2013) 1.63
Synthetic prions generated in vitro are similar to a newly identified subpopulation of PrPSc from sporadic Creutzfeldt-Jakob Disease. Protein Sci (2005) 1.62
Continuous quinacrine treatment results in the formation of drug-resistant prions. PLoS Pathog (2009) 1.58
Genetics and molecular pathogenesis of sporadic and hereditary cerebral amyloid angiopathies. Acta Neuropathol (2009) 1.57
Copper and the prion protein: methods, structures, function, and disease. Annu Rev Phys Chem (2007) 1.56
Protection from cytosolic prion protein toxicity by modulation of protein translocation. EMBO J (2004) 1.55
Discovery of 2-aminothiazoles as potent antiprion compounds. J Virol (2009) 1.51
Evaluation of potential infectivity of Alzheimer and Parkinson disease proteins in recipients of cadaver-derived human growth hormone. JAMA Neurol (2013) 1.42
PrPTSE distribution in a primate model of variant, sporadic, and iatrogenic Creutzfeldt-Jakob disease. J Virol (2005) 1.33
The crucial role of metal ions in neurodegeneration: the basis for a promising therapeutic strategy. Br J Pharmacol (2005) 1.32
Proteopathic tau seeding predicts tauopathy in vivo. Proc Natl Acad Sci U S A (2014) 1.28
Immunization delays the onset of prion disease in mice. Am J Pathol (2002) 1.26
Cell division modulates prion accumulation in cultured cells. Proc Natl Acad Sci U S A (2007) 1.25
Neurogenetics: advancing the "next-generation" of brain research. Neuron (2010) 1.16
Protein aggregate spreading in neurodegenerative diseases: problems and perspectives. Neurosci Res (2011) 1.15
Protease-sensitive conformers in broad spectrum of distinct PrPSc structures in sporadic Creutzfeldt-Jakob disease are indicator of progression rate. PLoS Pathog (2011) 1.11
Drug resistance confounding prion therapeutics. Proc Natl Acad Sci U S A (2013) 1.10
Human prion strain selection in transgenic mice. Ann Neurol (2010) 1.06
Creutzfeldt-Jacob Disease: a case report. Cases J (2008) 1.05
Dichotomous versus palm-type mechanisms of lateral assembly of amyloid fibrils. Protein Sci (2006) 1.04
The peculiar nature of unfolding of the human prion protein. Protein Sci (2004) 1.04
Early onset prion disease from octarepeat expansion correlates with copper binding properties. PLoS Pathog (2009) 1.03
A regulatory role of the Rnq1 nonprion domain for prion propagation and polyglutamine aggregates. Mol Cell Biol (2008) 1.03
Human prion disease and relative risk associated with chronic wasting disease. Emerg Infect Dis (2006) 1.02
Human prion proteins with pathogenic mutations share common conformational changes resulting in enhanced binding to glycosaminoglycans. Proc Natl Acad Sci U S A (2007) 1.02
Analysis of PRNP gene codon 129 polymorphism in the Greek population. Eur J Epidemiol (2006) 1.01
Cellular mechanisms of protein aggregate propagation. Curr Opin Neurol (2012) 1.01
The polybasic N-terminal region of the prion protein controls the physical properties of both the cellular and fibrillar forms of PrP. J Mol Biol (2008) 1.01
Anti-PrP Mab 6D11 suppresses PrP(Sc) replication in prion infected myeloid precursor line FDC-P1/22L and in the lymphoreticular system in vivo. Neurobiol Dis (2009) 1.00
Dynamic diagnosis of familial prion diseases supports the β2-α2 loop as a universal interference target. PLoS One (2011) 0.98
Convergent replication of mouse synthetic prion strains. Am J Pathol (2013) 0.98
Chemical induction of misfolded prion protein conformers in cell culture. J Biol Chem (2009) 0.97
Tau and 14-3-3 of genetic and sporadic Creutzfeldt-Jakob disease patients in Israel. J Neurol (2010) 0.96
The efficacy of tetracyclines in peripheral and intracerebral prion infection. PLoS One (2008) 0.96
Characterization of the amyloid bacterial inclusion bodies of the HET-s fungal prion. Microb Cell Fact (2009) 0.95
Context dependent neuroprotective properties of prion protein (PrP). Prion (2009) 0.95
Aggregated proteins in schizophrenia and other chronic mental diseases: DISC1opathies. Prion (2012) 0.93
Iron and iron/manganese ratio in forage from Icelandic sheep farms: relation to scrapie. Acta Vet Scand (2006) 0.93
Targeting intrinsically disordered proteins in neurodegenerative and protein dysfunction diseases: another illustration of the D(2) concept. Expert Rev Proteomics (2010) 0.93
Proteomics of dense core secretory vesicles reveal distinct protein categories for secretion of neuroeffectors for cell-cell communication. J Proteome Res (2010) 0.93
Pharmacokinetics and metabolism of 2-aminothiazoles with antiprion activity in mice. Pharm Res (2013) 0.92
Possible role of region 152-156 in the structural duality of a peptide fragment from sheep prion protein. Protein Sci (2004) 0.90
Allelic origin of protease-sensitive and protease-resistant prion protein isoforms in Gerstmann-Sträussler-Scheinker disease with the P102L mutation. PLoS One (2012) 0.90
Prion protein and copper cooperatively protect neurons by modulating NMDA receptor through S-nitrosylation. Antioxid Redox Signal (2015) 0.90
Clinical trials for prion disease: difficult challenges, but hope for the future. Lancet Neurol (2009) 0.90
Tissue distribution, biochemical properties, and transmission of mouse type A AApoAII amyloid fibrils. Am J Pathol (2004) 0.90
Enhanced correction methods for hydrogen exchange-mass spectrometric studies of amyloid fibrils. Protein Sci (2003) 0.89
Tau phosphorylation, aggregation, and cell toxicity. J Biomed Biotechnol (2006) 0.89
Nonpolar substitution at the C-terminus of the prion protein, a mimic of the glycosylphosphatidylinositol anchor, partially impairs amyloid fibril formation. Biochemistry (2007) 0.89
Novel antibody derivatives for proteome and high-content analysis. Anal Bioanal Chem (2010) 0.88
Structural dynamics of the ΔE22 (Osaka) familial Alzheimer's disease-linked amyloid β-protein. Amyloid (2011) 0.87
A survey of antiprion compounds reveals the prevalence of non-PrP molecular targets. J Biol Chem (2011) 0.86
Identification of I137M and other mutations that modulate incubation periods for two human prion strains. J Virol (2012) 0.86
Molecular pathogenesis of sporadic prion diseases in man. Prion (2012) 0.85
Misassembly of full-length Disrupted-in-Schizophrenia 1 protein is linked to altered dopamine homeostasis and behavioral deficits. Mol Psychiatry (2016) 0.85
Intrinsically disordered proteins and their (disordered) proteomes in neurodegenerative disorders. Front Aging Neurosci (2015) 0.85
Single-chain fragment variable passive immunotherapies for neurodegenerative diseases. Int J Mol Sci (2013) 0.84
Styryl-based and tricyclic compounds as potential anti-prion agents. PLoS One (2011) 0.83
Prion protein and aging. Front Cell Dev Biol (2014) 0.83
Role of prion protein aggregation in neurotoxicity. Int J Mol Sci (2012) 0.83
Co-existence of distinct prion types enables conformational evolution of human PrPSc by competitive selection. J Biol Chem (2013) 0.83
Immunomodulation for prion and prion-related diseases. Expert Rev Vaccines (2010) 0.80
Creutzfeldt-Jakob disease with E200K PRNP mutation: a case report and revision of the literature. Neurol Sci (2009) 0.80
Heightened incidence of sporadic Creutzfeldt-Jakob disease is associated with a shift in clinicopathological profiles. J Neurol (2008) 0.80
Calcium binding promotes prion protein fragment 90-231 conformational change toward a membrane destabilizing and cytotoxic structure. PLoS One (2012) 0.80
Mad cow and other maladies: update on emerging infectious diseases. Proc (Bayl Univ Med Cent) (2004) 0.80
A bipolar functionality of Q/N-rich proteins: Lsm4 amyloid causes clearance of yeast prions. Microbiologyopen (2013) 0.80
Successes and challenges in phenotype-based lead discovery for prion diseases. J Med Chem (2014) 0.79
On the intrinsic disorder status of the major players in programmed cell death pathways. F1000Res (2013) 0.79
Amyotrophic lateral sclerosis and organ donation: is there risk of disease transmission? Ann Neurol (2012) 0.79
Antiprion compounds that reduce PrP(Sc) levels in dividing and stationary-phase cells. Bioorg Med Chem (2013) 0.78
A case of gerstmann-sträussler-scheinker disease. J Clin Neurol (2010) 0.78
Computational and experimental studies on β-sheet breakers targeting Aβ1-40 fibrils. J Biol Chem (2014) 0.78
Optimization of Arylamides as Novel, Potent and Brain-penetrant Antiprion Lead Compounds. ACS Med Chem Lett (2013) 0.78
Pros and cons of a prion-like pathogenesis in Parkinson's disease. BMC Neurol (2011) 0.78
The structure of human prions: from biology to structural models-considerations and pitfalls. Viruses (2014) 0.78
Failure of prion protein oxidative folding guides the formation of toxic transmembrane forms. J Biol Chem (2012) 0.78
Grass plants bind, retain, uptake, and transport infectious prions. Cell Rep (2015) 0.77
Molecular genealogy tools for white-tailed deer with chronic wasting disease. Can J Vet Res (2010) 0.77
Prion protein paralog doppel protein interacts with alpha-2-macroglobulin: a plausible mechanism for doppel-mediated neurodegeneration. PLoS One (2009) 0.77
Early behavioral changes and quantitative analysis of neuropathological features in murine prion disease: stereological analysis in the albino Swiss mice model. Prion (2011) 0.77
Copper-induced structural propensities of the amyloidogenic region of human prion protein. J Biol Inorg Chem (2014) 0.77
Immune-directed gene therapeutic development for Alzheimer's, prion, and Parkinson's diseases. J Neuroimmune Pharmacol (2008) 0.77
Infection as a cause of multiple sclerosis. BMJ (2002) 0.77
Cognitive aging and Alzheimer's disease. Postgrad Med J (2005) 0.77