Published in Lancet on January 15, 2000
De novo designed peptide-based amyloid fibrils. Proc Natl Acad Sci U S A (2002) 2.59
Protein aggregation diseases: pathogenicity and therapeutic perspectives. Nat Rev Drug Discov (2010) 2.58
Scrapie prion protein accumulation by scrapie-infected neuroblastoma cells abrogated by exposure to a prion protein antibody. Proc Natl Acad Sci U S A (2001) 2.53
Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein. EMBO J (2003) 2.34
Dominant-negative inhibition of prion replication in transgenic mice. Proc Natl Acad Sci U S A (2002) 1.65
Evaluation of quinacrine treatment for prion diseases. J Virol (2003) 1.59
Pharmacological chaperone-mediated in vivo folding and stabilization of the P23H-opsin mutant associated with autosomal dominant retinitis pigmentosa. J Biol Chem (2003) 1.51
Mimicking dominant negative inhibition of prion replication through structure-based drug design. Proc Natl Acad Sci U S A (2000) 1.41
The prion strain phenomenon: molecular basis and unprecedented features. Biochim Biophys Acta (2006) 1.39
Tetracyclines affect prion infectivity. Proc Natl Acad Sci U S A (2002) 1.39
Potent inhibition of scrapie prion replication in cultured cells by bis-acridines. Proc Natl Acad Sci U S A (2003) 1.32
Branched polyamines cure prion-infected neuroblastoma cells. J Virol (2001) 1.32
Immunization delays the onset of prion disease in mice. Am J Pathol (2002) 1.26
Antihypertensive drug guanabenz is active in vivo against both yeast and mammalian prions. PLoS One (2008) 1.13
Prion protein misfolding affects calcium homeostasis and sensitizes cells to endoplasmic reticulum stress. PLoS One (2010) 1.11
Progress and problems in the biology, diagnostics, and therapeutics of prion diseases. J Clin Invest (2004) 1.09
Importance of N-glycosylation positioning for cell-surface expression, targeting, affinity and quality control of the human AT1 receptor. Biochem J (2005) 1.03
Recent advances in prion chemotherapeutics. Infect Disord Drug Targets (2009) 1.03
Cross currents in protein misfolding disorders: interactions and therapy. CNS Neurol Disord Drug Targets (2009) 0.98
Trafficking and quality control of the gonadotropin releasing hormone receptor in health and disease. Mol Cell Endocrinol (2008) 0.98
Transport of prion protein across the blood-brain barrier. Exp Neurol (2009) 0.95
Chaperoning G protein-coupled receptors: from cell biology to therapeutics. Endocr Rev (2014) 0.91
Evidence that the 127-164 region of prion proteins has two equi-energetic conformations with beta or alpha features. Biophys J (2001) 0.90
Therapeutic rescue of misfolded mutants: validation of primary high throughput screens for identification of pharmacoperone drugs. PLoS One (2011) 0.89
Preventing beta-amyloid fibrillization and deposition: beta-sheet breakers and pathological chaperone inhibitors. BMC Neurosci (2008) 0.86
Beta-amyloid production, aggregation, and clearance as targets for therapy in Alzheimer's disease. Cell Mol Neurobiol (2002) 0.86
Peptide- and saccharide-conjugated dendrimers for targeted drug delivery: a concise review. Interface Focus (2012) 0.82
Uncovering the Mechanism of Aggregation of Human Transthyretin. J Biol Chem (2015) 0.82
Alpha synuclein is transported into and out of the brain by the blood-brain barrier. Peptides (2014) 0.81
Transitioning pharmacoperones to therapeutic use: in vivo proof-of-principle and design of high throughput screens. Pharmacol Res (2013) 0.81
Enhanced stability of human prion proteins with two disulfide bridges. Biophys J (2006) 0.79
Disease-associated prion protein elicits immunoglobulin M responses in vivo. Mol Med (2006) 0.79
Escorts take the lead molecular chaperones as therapeutic targets. Prog Mol Biol Transl Sci (2010) 0.79
Assay strategies for identification of therapeutic leads that target protein trafficking. Trends Pharmacol Sci (2015) 0.78
Inoculation of scrapie with the self-assembling RADA-peptide disrupts prion accumulation and extends hamster survival. PLoS One (2009) 0.78
The suppression of prion propagation using poly-L-lysine by targeting plasminogen that stimulates prion protein conversion. Biomaterials (2011) 0.76
A phenotypic high throughput screening assay for the identification of pharmacoperones for the gonadotropin releasing hormone receptor. Assay Drug Dev Technol (2014) 0.76
Vitamin D 2 interacts with Human PrP(c) (90-231) and breaks PrP(c) oligomerization in vitro. Prion (2013) 0.75
Recent developments in the pathogenesis, diagnosis, and therapy of prion diseases. Dialogues Clin Neurosci (2001) 0.75
Prions: Beyond a Single Protein. Clin Microbiol Rev (2016) 0.75
Prion-like characteristics of the bacterial protein Microcin E492. Sci Rep (2017) 0.75
Amyloid beta: structure, biology and structure-based therapeutic development. Acta Pharmacol Sin (2017) 0.75
Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD. Nature (1996) 14.40
Mouse polyclonal and monoclonal antibody to scrapie-associated fibril proteins. J Virol (1987) 7.05
Production of the Alzheimer amyloid beta protein by normal proteolytic processing. Science (1992) 5.22
Clearance of Alzheimer's amyloid-ss(1-40) peptide from brain by LDL receptor-related protein-1 at the blood-brain barrier. J Clin Invest (2000) 5.07
Mutation of the Alzheimer's disease amyloid gene in hereditary cerebral hemorrhage, Dutch type. Science (1990) 4.71
Apolipoprotein E: a pathological chaperone protein in patients with cerebral and systemic amyloid. Neurosci Lett (1992) 4.29
Diagnosis of new variant Creutzfeldt-Jakob disease by tonsil biopsy. Lancet (1997) 4.28
Beta-sheet breaker peptides inhibit fibrillogenesis in a rat brain model of amyloidosis: implications for Alzheimer's therapy. Nat Med (1998) 4.17
a controlled trial of intratumoral ONYX-015, a selectively-replicating adenovirus, in combination with cisplatin and 5-fluorouracil in patients with recurrent head and neck cancer. Nat Med (2000) 4.15
The amino acid sequence of a major nonimmunoglobulin component of some amyloid fibrils. J Clin Invest (1972) 3.96
An Actinophage for Streptomyces griseus. J Bacteriol (1947) 3.95
Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. Proc Natl Acad Sci U S A (1999) 3.43
Neurotoxicity of a prion protein fragment. Nature (1993) 3.38
Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker's syndrome. Exp Neurol (1989) 3.00
Regeneration of amino acids from thiazolinones formed in the Edman degradation. Anal Biochem (1975) 2.86
Molecular assessment of the potential transmissibilities of BSE and scrapie to humans. Nature (1997) 2.80
Glycoprotein 330/megalin: probable role in receptor-mediated transport of apolipoprotein J alone and in a complex with Alzheimer disease amyloid beta at the blood-brain and blood-cerebrospinal fluid barriers. Proc Natl Acad Sci U S A (1996) 2.79
Down patients: extracellular preamyloid deposits precede neuritic degeneration and senile plaques. Neurosci Lett (1989) 2.69
Acceleration of Alzheimer's fibril formation by apolipoprotein E in vitro. Am J Pathol (1994) 2.66
The cerebrospinal-fluid soluble form of Alzheimer's amyloid beta is complexed to SP-40,40 (apolipoprotein J), an inhibitor of the complement membrane-attack complex. Biochem J (1993) 2.66
Structural studies of immunoglobulin G. Nature (1969) 2.63
Alzheimer neurofibrillary tangles: monoclonal antibodies to inherent antigen(s). Acta Neuropathol (1984) 2.58
Variations in the S-S bridges of immunoglobins G: interchain disulfide bridges of gamma G3 myeloma proteins. J Mol Biol (1968) 2.57
Sequence of deposition of heterogeneous amyloid beta-peptides and APO E in Down syndrome: implications for initial events in amyloid plaque formation. Neurobiol Dis (1996) 2.50
Neuropathological changes in scrapie and Alzheimer's disease are associated with increased expression of apolipoprotein E and cathepsin D in astrocytes. J Virol (1991) 2.47
Copper stimulates endocytosis of the prion protein. J Biol Chem (1998) 2.47
Glycolipid-anchored proteins in neuroblastoma cells form detergent-resistant complexes without caveolin. J Cell Biol (1995) 2.46
A stop-codon mutation in the BRI gene associated with familial British dementia. Nature (1999) 2.46
Human amyloidosis, Alzheimer disease and related disorders. Lab Invest (1988) 2.44
Neurological illness in transgenic mice expressing a prion protein with an insertional mutation. Neuron (1998) 2.40
Infection-specific particle from the unconventional slow virus diseases. Science (1984) 2.34
Sporadic Creutzfeldt-Jakob disease: co-occurrence of different types of PrP(Sc) in the same brain. Neurology (1999) 2.33
BSE transmission to macaques. Nature (1996) 2.32
The disulphide bonds of the heavy chain of rabbit immunoglobulin G. Biochem J (1970) 2.32
Alpha chain disease. Ann N Y Acad Sci (1971) 2.31
Preamyloid deposits in the cerebral cortex of patients with Alzheimer's disease and nondemented individuals. Neurosci Lett (1988) 2.26
Differences between vascular and plaque core amyloid in Alzheimer's disease. J Neurochem (1988) 2.24
Successful transmission of three mouse-adapted scrapie strains to murine neuroblastoma cell lines overexpressing wild-type mouse prion protein. J Virol (2000) 2.18
In vitro formation of amyloid fibrils from two synthetic peptides of different lengths homologous to Alzheimer's disease beta-protein. Biochem Biophys Res Commun (1986) 2.16
A comparison of the replication cycles of simian virus 40 in human diploid and African green monkey kidney cells. Virology (1966) 2.13
Systematic review and meta-analysis of intraoperative peritoneal lavage for colorectal cancer staging. Br J Surg (2013) 2.12
A decamer duplication in the 3' region of the BRI gene originates an amyloid peptide that is associated with dementia in a Danish kindred. Proc Natl Acad Sci U S A (2000) 2.11
Growth of murine cytomegalovirus in various cell lines. J Virol (1971) 2.06
Cellular isoform of the scrapie agent protein participates in lymphocyte activation. Cell (1990) 2.06
Isolation and partial characterization of SAA-an amyloid-related protein from human serum. J Immunol (1976) 2.05
Megalin knockout mice as an animal model of low molecular weight proteinuria. Am J Pathol (1999) 2.03
Bovine viral diarrhea virus NS3 serine proteinase: polyprotein cleavage sites, cofactor requirements, and molecular model of an enzyme essential for pestivirus replication. J Virol (1997) 2.00
Apolipoprotein J (clusterin) and Alzheimer's disease. Microsc Res Tech (2000) 2.00
Nearly ubiquitous tissue distribution of the scrapie agent precursor protein. Neurology (1992) 2.00
The interactions of coupling ATPases with nucleotides. Biochim Biophys Acta (1978) 1.99
Fate of cerebrospinal fluid-borne amyloid beta-peptide: rapid clearance into blood and appreciable accumulation by cerebral arteries. J Neurochem (1996) 1.96
A single amino acid alteration (101L) introduced into murine PrP dramatically alters incubation time of transmissible spongiform encephalopathy. EMBO J (1999) 1.96
Immunological comparison of scrapie-associated fibrils isolated from animals infected with four different scrapie strains. J Virol (1986) 1.95
Boundary lubrication by lubricin is mediated by O-linked beta(1-3)Gal-GalNAc oligosaccharides. Glycoconj J (2001) 1.95
Vascular variant of prion protein cerebral amyloidosis with tau-positive neurofibrillary tangles: the phenotype of the stop codon 145 mutation in PRNP. Proc Natl Acad Sci U S A (1996) 1.94
The inactivation of simian virus 40 infectivity and antigen-inducing capacity by ultraviolet light. Virology (1965) 1.93
Anti-Aβ autoantibodies in the CSF of a patient with CAA-related inflammation: a case report. Neurology (2011) 1.91
NEOMYCIN-PRODUCTION AND ANTIBIOTIC PROPERTIES. J Clin Invest (1949) 1.90
Molecular location of a species-specific epitope on the hamster scrapie agent protein. J Virol (1991) 1.89
Preferential association of kappa IIIb light chains with monoclonal human IgM kappa autoantibodies. J Immunol (1983) 1.87
A 25-kDa NH2-terminal fragment carries all the antibacterial activities of the human neutrophil 60-kDa bactericidal/permeability-increasing protein. J Biol Chem (1987) 1.86
Lipidation of apolipoprotein E influences its isoform-specific interaction with Alzheimer's amyloid beta peptides. Biochem J (2000) 1.84
The primary structure of human serum transferrin. The structures of seven cyanogen bromide fragments and the assembly of the complete structure. J Biol Chem (1983) 1.83
Primary structure of the "hinge" region of human IgG3. Probable quadruplication of a 15-amino acid residue basic unit. J Biol Chem (1977) 1.83
Apolipoprotein E: binding to soluble Alzheimer's beta-amyloid. Biochem Biophys Res Commun (1993) 1.80
In vitro replication of scrapie agent in a neuronal model: infection of PC12 cells. J Gen Virol (1984) 1.80
Phenotypic variability of Gerstmann-Sträussler-Scheinker disease is associated with prion protein heterogeneity. J Neuropathol Exp Neurol (1998) 1.79
Amyloid fibrils in hereditary cerebral hemorrhage with amyloidosis of Icelandic type is a variant of gamma-trace basic protein (cystatin C). Proc Natl Acad Sci U S A (1986) 1.78
The effect of actinomycin D on the transcription and replication of simian virus 40 deoxyribonucleic acid. Virology (1969) 1.78
Biochemical differences among scrapie-associated fibrils support the biological diversity of scrapie agents. J Gen Virol (1985) 1.75
Isolation of a cDNA clone encoding the leader peptide of prion protein and expression of the homologous gene in various tissues. Proc Natl Acad Sci U S A (1986) 1.75
Antiviral activity of arildone on deoxyribonucleic acid and ribonucleic acid viruses. Antimicrob Agents Chemother (1980) 1.75
A glycolipid-anchored prion protein is endocytosed via clathrin-coated pits. J Cell Biol (1994) 1.74
Amyloid protein of Gerstmann-Sträussler-Scheinker disease (Indiana kindred) is an 11 kd fragment of prion protein with an N-terminal glycine at codon 58. EMBO J (1991) 1.74
Inhibition of Alzheimer's amyloidosis by peptides that prevent beta-sheet conformation. Biochem Biophys Res Commun (1996) 1.73
Nucleotide sequence of the small double-stranded RNA segment of bacteriophage phi 6: novel mechanism of natural translational control. J Virol (1986) 1.72
Fibrillogenesis in Alzheimer's disease of amyloid beta peptides and apolipoprotein E. Biochem J (1995) 1.72
Substitutions at codon 22 of Alzheimer's abeta peptide induce diverse conformational changes and apoptotic effects in human cerebral endothelial cells. J Biol Chem (2000) 1.71
Isolation and characterization of amyloid P component from Alzheimer's disease and other types of cerebral amyloidosis. Lab Invest (1988) 1.70
Amyloid fibrils in Gerstmann-Sträussler-Scheinker disease (Indiana and Swedish kindreds) express only PrP peptides encoded by the mutant allele. Cell (1994) 1.69
Immunization with a nontoxic/nonfibrillar amyloid-beta homologous peptide reduces Alzheimer's disease-associated pathology in transgenic mice. Am J Pathol (2001) 1.69
Synthetic peptides homologous to prion protein residues 106-147 form amyloid-like fibrils in vitro. Proc Natl Acad Sci U S A (1993) 1.68
Detection of scrapie-associated fibril (SAF) proteins using anti-SAF antibody in non-purified tissue preparations. J Gen Virol (1986) 1.67