Published in J Biol Chem on October 31, 1997
Lysosomotropic agents and cysteine protease inhibitors inhibit scrapie-associated prion protein accumulation. J Virol (2000) 2.15
From conversion to aggregation: protofibril formation of the prion protein. Proc Natl Acad Sci U S A (2004) 2.14
Sulfated glycans and elevated temperature stimulate PrP(Sc)-dependent cell-free formation of protease-resistant prion protein. EMBO J (2001) 1.95
Prion protein expression in different species: analysis with a panel of new mAbs. Proc Natl Acad Sci U S A (1998) 1.80
Selective incorporation of polyanionic molecules into hamster prions. J Biol Chem (2007) 1.71
A scrapie-like unfolding intermediate of the prion protein domain PrP(121-231) induced by acidic pH. Proc Natl Acad Sci U S A (1998) 1.55
The divergence, actions, roles, and relatives of sodium-coupled bicarbonate transporters. Physiol Rev (2013) 1.50
The rate and equilibrium constants for a multistep reaction sequence for the aggregation of superoxide dismutase in amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2004) 1.46
Mapping the early steps in the pH-induced conformational conversion of the prion protein. Proc Natl Acad Sci U S A (2001) 1.28
Pauling and Corey's alpha-pleated sheet structure may define the prefibrillar amyloidogenic intermediate in amyloid disease. Proc Natl Acad Sci U S A (2004) 1.23
An aggregation-specific enzyme-linked immunosorbent assay: detection of conformational differences between recombinant PrP protein dimers and PrP(Sc) aggregates. J Virol (2005) 1.22
Influence of the N-terminal domain on the aggregation properties of the prion protein. Protein Sci (2005) 1.16
Cytolytic toxin Cyt1A and its mechanism of membrane damage: data and hypotheses. Appl Environ Microbiol (2003) 1.15
Pathogenic mutations in the hydrophobic core of the human prion protein can promote structural instability and misfolding. J Mol Biol (2010) 1.08
The peculiar nature of unfolding of the human prion protein. Protein Sci (2004) 1.04
Prion protein amyloid formation under native-like conditions involves refolding of the C-terminal alpha-helical domain. J Biol Chem (2008) 1.02
Proton binding to proteins: a free-energy component analysis using a dielectric continuum model. Biophys J (2005) 1.02
Role of the single disulphide bond of beta(2)-microglobulin in amyloidosis in vitro. Protein Sci (2001) 0.99
Prion proteins with pathogenic and protective mutations show similar structure and dynamics. Biochemistry (2009) 0.97
Influence of pH on the human prion protein: insights into the early steps of misfolding. Biophys J (2010) 0.93
Probing the role of PrP repeats in conformational conversion and amyloid assembly of chimeric yeast prions. J Biol Chem (2007) 0.92
Molecular dynamics simulation of dimeric and monomeric forms of human prion protein: insight into dynamics and properties. Biophys J (2003) 0.90
Aggregation of prion protein with insertion mutations is proportional to the number of inserts. Biochem J (2007) 0.90
Chemical chaperone and inhibitor discovery: potential treatments for protein conformational diseases. Perspect Medicin Chem (2007) 0.90
Checking the pH-induced conformational transition of prion protein by molecular dynamics simulations: effect of protonation of histidine residues. Biophys J (2004) 0.90
Characterization of cell-surface prion protein relative to its recombinant analogue: insights from molecular dynamics simulations of diglycosylated, membrane-bound human prion protein. J Neurochem (2009) 0.89
Copper induces increased beta-sheet content in the scrapie-susceptible ovine prion protein PrPVRQ compared with the resistant allelic variant PrPARR. Biochem J (2004) 0.89
The molecular pathology of CJD: old and new variants. Mol Pathol (2001) 0.89
Methionine oxidation perturbs the structural core of the prion protein and suggests a generic misfolding pathway. J Biol Chem (2012) 0.88
Conformational pH dependence of intermediate states during oligomerization of the human prion protein. Protein Sci (2008) 0.88
NMR characterization of the pH 4 beta-intermediate of the prion protein: the N-terminal half of the protein remains unstructured and retains a high degree of flexibility. Biochem J (2007) 0.87
Stability and conformational properties of doppel, a prion-like protein, and its single-disulphide mutant. Biochem J (2003) 0.86
Human PrP90-231-induced cell death is associated with intracellular accumulation of insoluble and protease-resistant macroaggregates and lysosomal dysfunction. Cell Death Dis (2011) 0.86
Role of prion protein aggregation in neurotoxicity. Int J Mol Sci (2012) 0.83
Misfolding pathways of the prion protein probed by molecular dynamics simulations. Biophys J (2004) 0.83
Structural and dynamic properties of the human prion protein. Biophys J (2014) 0.82
Synthetic Aβ peptides acquire prion-like properties in the brain. Oncotarget (2015) 0.82
Isolation and characterization of a polymerized prion protein. Biochem J (2002) 0.81
Phosphorylation of prion protein at serine 43 induces prion protein conformational change. J Neurosci (2009) 0.80
Calcium binding promotes prion protein fragment 90-231 conformational change toward a membrane destabilizing and cytotoxic structure. PLoS One (2012) 0.80
Proper calibration of ultrasonic power enabled the quantitative analysis of the ultrasonication-induced amyloid formation process. Protein Sci (2011) 0.80
Acid-induced molten globule state of a prion protein: crucial role of Strand 1-Helix 1-Strand 2 segment. J Biol Chem (2014) 0.79
Similar folds with different stabilization mechanisms: the cases of Prion and Doppel proteins. BMC Struct Biol (2006) 0.79
Enhanced stability of human prion proteins with two disulfide bridges. Biophys J (2006) 0.79
Two misfolding routes for the prion protein around pH 4.5. PLoS Comput Biol (2013) 0.78
N-terminal helix-cap in α-helix 2 modulates β-state misfolding in rabbit and hamster prion proteins. PLoS One (2013) 0.78
A Native-like Intermediate Serves as a Branching Point between the Folding and Aggregation Pathways of the Mouse Prion Protein. Structure (2015) 0.78
Disruption of the X-loop turn of the prion protein linked to scrapie resistance. Protein Eng Des Sel (2012) 0.78
Role of lipid in forming an infectious prion? Acta Biochim Biophys Sin (Shanghai) (2013) 0.78
Nucleic acid induced unfolding of recombinant prion protein globular fragment is pH dependent. Protein Sci (2014) 0.77
Thermodynamic characterization of the unfolding of the prion protein. Biophys J (2014) 0.77
Partially Unfolded Forms of the Prion Protein Populated under Misfolding-promoting Conditions: CHARACTERIZATION BY HYDROGEN EXCHANGE MASS SPECTROMETRY AND NMR. J Biol Chem (2015) 0.77
Assessment of the PrPc Amino-Terminal Domain in Prion Species Barriers. J Virol (2016) 0.77
Rapid folding of the prion protein captured by pressure-jump. Eur Biophys J (2009) 0.76
Polymorphisms at amino acid residues 141 and 154 influence conformational variation in ovine PrP. Biomed Res Int (2014) 0.75
Unique Properties of the Rabbit Prion Protein Oligomer. PLoS One (2016) 0.75
Unique structural properties associated with mouse prion Δ105-125 protein. Prion (2013) 0.75
Molecular dynamics simulations capture the misfolding of the bovine prion protein at acidic pH. Biomolecules (2014) 0.75
Cyclin-dependent kinase 5 phosphorylation of familial prion protein mutants exacerbates conversion into amyloid structure. J Biol Chem (2015) 0.75
A general approach to renaturation of recombinant proteins produced as inclusion bodies. Dokl Biochem Biophys (2001) 0.75
Molecular dynamics simulation of temperature induced unfolding of animal prion protein. J Mol Model (2013) 0.75
Simulations of Membrane-bound Diglycosylated Human Prion Protein Reveal Potential Protective Mechanisms against Misfolding. J Neurochem (2017) 0.75
The intrinsic helical propensities of the helical fragments in prion protein under neutral and low pH conditions: a replica exchange molecular dynamics study. J Mol Model (2013) 0.75
Enhanced sensitivity of PTEN-deficient tumors to inhibition of FRAP/mTOR. Proc Natl Acad Sci U S A (2001) 6.99
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
Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol (1996) 4.10
Prediction of the risk of hereditary retinoblastoma, using DNA polymorphisms within the retinoblastoma gene. N Engl J Med (1988) 3.90
Truncated forms of the human prion protein in normal brain and in prion diseases. J Biol Chem (1995) 3.20
Screening for intimate partner violence by health care providers. Barriers and interventions. Am J Prev Med (2000) 3.04
Oncogenic point mutations in the human retinoblastoma gene: their application to genetic counseling. N Engl J Med (1989) 3.03
Paired helical filaments from Alzheimer disease patients contain cytoskeletal components. Proc Natl Acad Sci U S A (1985) 2.86
Molecular assessment of the potential transmissibilities of BSE and scrapie to humans. Nature (1997) 2.80
Presence of soluble amyloid beta-peptide precedes amyloid plaque formation in Down's syndrome. Nat Med (1996) 2.71
Homozygosity of chromosome 13 in retinoblastoma. N Engl J Med (1984) 2.49
Genetic influence on the structural variations of the abnormal prion protein. Proc Natl Acad Sci U S A (2000) 2.46
Fibril formation by primate, rodent, and Dutch-hemorrhagic analogues of Alzheimer amyloid beta-protein. Biochemistry (1992) 2.36
pH-dependent structural transitions of Alzheimer amyloid peptides. Biophys J (1991) 2.33
Immunohistochemical localization of glial fibrillary acidic protein in human glial neoplasms. Cancer (1980) 2.26
Parental origin of mutations of the retinoblastoma gene. Nature (1989) 2.26
Crystal structure of the human prion protein reveals a mechanism for oligomerization. Nat Struct Biol (2001) 2.19
Morvan's syndrome: peripheral and central nervous system and cardiac involvement with antibodies to voltage-gated potassium channels. Brain (2001) 2.14
The widespread alteration of neurites in Alzheimer's disease may be unrelated to amyloid deposition. Ann Neurol (1989) 2.07
Ubiquitin is associated with abnormal cytoplasmic filaments characteristic of neurodegenerative diseases. Proc Natl Acad Sci U S A (1988) 2.04
A novel phenotype in familial Creutzfeldt-Jakob disease: prion protein gene E200K mutation coupled with valine at codon 129 and type 2 protease-resistant prion protein. Ann Neurol (1999) 1.98
Presenilin-1 mutations in Alzheimer's disease. Nature (2000) 1.97
Different patterns of truncated prion protein fragments correlate with distinct phenotypes in P102L Gerstmann-Sträussler-Scheinker disease. Proc Natl Acad Sci U S A (1998) 1.85
Membrane environment alters the conformational structure of the recombinant human prion protein. J Biol Chem (1999) 1.83
Typing prion isoforms. Nature (1997) 1.82
Prion protein expression in different species: analysis with a panel of new mAbs. Proc Natl Acad Sci U S A (1998) 1.80
Parkinson disease, dementia, and Alzheimer disease: clinicopathological correlations. Ann Neurol (1980) 1.78
Violence and reproductive health: current knowledge and future research directions. Matern Child Health J (2000) 1.77
Alpha-2 macroglobulin gene and Alzheimer disease. Nat Genet (1999) 1.73
Fatal familial insomnia and dysautonomia with selective degeneration of thalamic nuclei. N Engl J Med (1986) 1.73
Temperature-induced exposure of hydrophobic surfaces and its effect on the chaperone activity of alpha-crystallin. FEBS Lett (1995) 1.73
Neurofibrillary changes in human brain. An immunocytochemical study with a neurofilament antiserum. J Neuropathol Exp Neurol (1983) 1.72
Absence of protease-resistant prion protein in the cerebrospinal fluid of Creutzfeldt-Jakob disease. J Pathol (2001) 1.61
Bodian's silver method stains neurofilament polypeptides. Science (1981) 1.59
Clinical features of fatal familial insomnia: phenotypic variability in relation to a polymorphism at codon 129 of the prion protein gene. Brain Pathol (1998) 1.56
On the mechanism of alpha-helix to beta-sheet transition in the recombinant prion protein. Biochemistry (2001) 1.53
Aggregation and fibrillization of the recombinant human prion protein huPrP90-231. Biochemistry (2000) 1.52
Conformation and fibrillogenesis of Alzheimer A beta peptides with selected substitution of charged residues. J Mol Biol (1994) 1.52
Sensitivity of 14-3-3 protein test varies in subtypes of sporadic Creutzfeldt-Jakob disease. Neurology (2004) 1.49
Identification of an epitope in the C terminus of normal prion protein whose expression is modulated by binding events in the N terminus. J Mol Biol (2000) 1.44
Pregnant women's perspectives on intendedness of pregnancy. Womens Health Issues (1998) 1.43
Familial mutations and the thermodynamic stability of the recombinant human prion protein. J Biol Chem (1998) 1.43
Neuronal and microglial involvement in beta-amyloid protein deposition in Alzheimer's disease. Am J Pathol (1990) 1.41
Alteration of the serotonergic nervous system in fatal familial insomnia. Ann Neurol (2000) 1.41
Temperature-dependent chaperone activity and structural properties of human alphaA- and alphaB-crystallins. J Biol Chem (2000) 1.40
Phosphorylation of neurofilaments is altered in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol (1988) 1.40
Membrane binding induces destabilization of cytochrome c structure. Biochemistry (1991) 1.40
The prion protein has RNA binding and chaperoning properties characteristic of nucleocapsid protein NCP7 of HIV-1. J Biol Chem (2001) 1.38
Myoclonic epilepsy with lafora bodies. Some ultrastructural, histochemical, and biochemical aspects. Arch Neurol (1971) 1.36
Acceleration of amyloid fibril formation by specific binding of Abeta-(1-40) peptide to ganglioside-containing membrane vesicles. J Biol Chem (1997) 1.35
Amyloid precursor protein in senile plaques of Alzheimer disease. Lancet (1988) 1.33
Aberrant metal binding by prion protein in human prion disease. J Neurochem (2001) 1.33
Immunocytochemical properties of Alzheimer straight filaments. J Neurosci (1987) 1.32
Hirano body filaments contain actin and actin-associated proteins. J Neuropathol Exp Neurol (1987) 1.32
Nervous system in Pompe's disease. Ultrastructure and biochemistry. J Neuropathol Exp Neurol (1971) 1.31
Solution structure of the E200K variant of human prion protein. Implications for the mechanism of pathogenesis in familial prion diseases. J Biol Chem (2000) 1.30
Oxidative impairment in scrapie-infected mice is associated with brain metals perturbations and altered antioxidant activities. J Neurochem (2001) 1.30
Proteasomal degradation and N-terminal protease resistance of the codon 145 mutant prion protein. J Biol Chem (1999) 1.30
The chaperone protein BiP binds to a mutant prion protein and mediates its degradation by the proteasome. J Biol Chem (2000) 1.29
Fatal familial insomnia: a second kindred with mutation of prion protein gene at codon 178. Neurology (1992) 1.29
Soluble amyloid beta-protein is a marker of Alzheimer amyloid in brain but not in cerebrospinal fluid. Biochem Biophys Res Commun (1994) 1.27
Ultrastructural localization of beta-amyloid, tau, and ubiquitin epitopes in extracellular neurofibrillary tangles. Proc Natl Acad Sci U S A (1991) 1.26
Autonomic dysfunction in dementia with Lewy bodies. Neurology (2004) 1.25
Selective translation and degradation of heat-shock messenger RNAs in Drosophila. Enzyme (1990) 1.25
Prion protein aggregation reverted by low temperature in transfected cells carrying a prion protein gene mutation. J Biol Chem (1997) 1.24
Allelic origin of the abnormal prion protein isoform in familial prion diseases. Nat Med (1997) 1.23
Opposite roles of apolipoprotein E in normal brains and in Alzheimer's disease. Proc Natl Acad Sci U S A (1998) 1.22
Neuronal and astrocytic differentiation in human neuroepithelial neoplasms. An immunohistochemical study. J Neuropathol Exp Neurol (1983) 1.21
Aluminum effect on slow axonal transport: a novel impairment of neurofilament transport. J Neurosci (1984) 1.21
Puromycin: action on neuronal mitochondria. Science (1968) 1.20
Accessibility of a critical prion protein region involved in strain recognition and its implications for the early detection of prions. Cell Mol Life Sci (2008) 1.20
Reorganization of axoplasmic organelles following beta, beta'-iminodipropionitrile administration. J Cell Biol (1981) 1.19
Glial fibrillary acidic protein (GFAP) in ependymal cells during development. An immunocytochemical study. Brain Res (1980) 1.19
Creutzfeldt-Jakob disease in unusually young patients who consumed venison. Arch Neurol (2001) 1.18
A new type of late infantile amaurotic idiocy. The fine structure of the multilamellar cytosome. J Neuropathol Exp Neurol (1968) 1.18
Bacterial subretinal abscess: a case report and review of the literature. Am J Ophthalmol (2000) 1.17
Alz 50 recognizes abnormal filaments in Alzheimer's disease and progressive supranuclear palsy. Ann Neurol (1988) 1.17
The role of disulfide bridge in the folding and stability of the recombinant human prion protein. J Biol Chem (2000) 1.16
Effect of the D178N mutation and the codon 129 polymorphism on the metabolism of the prion protein. J Biol Chem (1996) 1.16
Hirano bodies contain tau protein. Brain Res (1987) 1.14
Pathological reaction of astrocytes in perinatal brain injury. Immunohistochemical study. Acta Neuropathol (1986) 1.13
Tau gene mutation in familial progressive subcortical gliosis. Nat Med (1999) 1.13
Human prion diseases. Curr Opin Neurol (1995) 1.13
The interaction between Alzheimer amyloid beta(1-40) peptide and ganglioside GM1-containing membranes. FEBS Lett (1997) 1.13
Ubiquitin is present on the cytokeratin intermediate filaments and Mallory bodies of hepatocytes. Lab Invest (1988) 1.12
Sensitivity and specificity of three clinical criteria for dementia with Lewy bodies in an autopsy-verified sample. Int J Geriatr Psychiatry (1999) 1.11
Familial prion disease with a novel 144-bp insertion in the prion protein gene in a Basque family. Neurology (1997) 1.11
Membrane interaction and conformational properties of the putative fusion peptide of PH-30, a protein active in sperm-egg fusion. Biochemistry (1994) 1.11
Heterogeneity of water-soluble amyloid beta-peptide in Alzheimer's disease and Down's syndrome brains. FEBS Lett (1997) 1.11
Transthyretin amyloidosis: a new mutation associated with dementia. Ann Neurol (1997) 1.10
A subtype of sporadic prion disease mimicking fatal familial insomnia. Neurology (1999) 1.10
Neuropil threads of Alzheimer's disease show a marked alteration of the normal cytoskeleton. J Neurosci (1991) 1.10