Stanley B. Prusiner
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Neurologist, biochemist
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Medical Biology
Stanley B. Prusiner's Degrees
- Masters Medicine University of Pennsylvania
- Bachelors Chemistry University of Pennsylvania
Why Is Stanley B. Prusiner Influential?
(Suggest an Edit or Addition)According to Wikipedia, Stanley Ben Prusiner is an American neurologist and biochemist. He is the director of the Institute for Neurodegenerative Diseases at University of California, San Francisco . Prusiner discovered prions, a class of infectious self-reproducing pathogens primarily or solely composed of protein. He received the Albert Lasker Award for Basic Medical Research in 1994 and the Nobel Prize in Physiology or Medicine in 1997 for prion research developed by him and his team of experts beginning in the early 1970s.
Stanley B. Prusiner's Published Works
Published Works
- Novel proteinaceous infectious particles cause scrapie. (1982) (4797)
- Nobel Lecture: Prions (1998) (4293)
- Molecular biology of prion diseases (1991) (1926)
- Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein (1992) (1654)
- A cellular gene encodes scrapie PrP 27-30 protein (1985) (1496)
- Identification of a protein that purifies with the scrapie prion. (1982) (1250)
- Eight prion strains have PrPSc molecules with different conformations (1998) (1242)
- Scrapie prion protein contains a phosphatidylinositol glycolipid (1987) (1094)
- Synthetic Mammalian Prions (2004) (1020)
- Scrapie prions aggregate to form amyloid-like birefringent rods (1983) (1004)
- Prion Protein Biology (1998) (907)
- Prion diseases and the BSE crisis. (1997) (890)
- A protease-resistant protein is a structural component of the Scrapie prion (1983) (890)
- Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication (1990) (875)
- Linkage of a prion protein missense variant to Gerstmann–Sträussler syndrome (1989) (864)
- Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein (1995) (856)
- Evidence for the Conformation of the Pathologic Isoform of the Prion Protein Enciphering and Propagating Prion Diversity (1996) (828)
- Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene (1986) (824)
- A transmembrane form of the prion protein in neurodegenerative disease. (1998) (735)
- Shattuck lecture--neurodegenerative diseases and prions. (2001) (721)
- Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques (1989) (705)
- Separation and properties of cellular and scrapie prion proteins. (1986) (624)
- Structure of the recombinant full-length hamster prion protein PrP(29-231): the N terminus is highly flexible. (1997) (622)
- Acridine and phenothiazine derivatives as pharmacotherapeutics for prion disease (2001) (604)
- Evidence for α-synuclein prions causing multiple system atrophy in humans with parkinsonism (2015) (580)
- Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans. (1999) (576)
- Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing. (1993) (574)
- Subcellular colocalization of the cellular and scrapie prion proteins in caveolae-like membranous domains. (1996) (539)
- Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel. (1999) (536)
- Spontaneous neurodegeneration in transgenic mice with mutant prion protein (1990) (535)
- Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation. (1997) (532)
- Antibodies inhibit prion propagation and clear cell cultures of prion infectivity (2001) (519)
- Pathologic conformations of prion proteins. (1998) (516)
- Copper binding to the prion protein: structural implications of four identical cooperative binding sites. (1999) (515)
- Prion protein selectively binds copper(II) ions. (1998) (510)
- Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells (1990) (506)
- Evidence for assembly of prions with left-handed β-helices into trimers (2004) (489)
- Structural clues to prion replication. (1994) (483)
- Distinct prion proteins in short and long scrapie incubation period mice (1987) (482)
- Evidence for synthesis of scrapie prion proteins in the endocytic pathway. (1992) (480)
- Formic acid pretreatment enhances immunostaining of cerebral and systemic amyloids. (1987) (480)
- Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies. (1993) (470)
- Purification and structural studies of a major scrapie prion protein (1984) (467)
- Further purification and characterization of scrapie prions. (1982) (459)
- A Unifying Role for Prions in Neurodegenerative Diseases (2012) (454)
- Solution structure of a 142-residue recombinant prion protein corresponding to the infectious fragment of the scrapie isoform. (1997) (438)
- Scrapie prion proteins are synthesized in neurons. (1986) (436)
- Cholesterol depletion and modification of COOH-terminal targeting sequence of the prion protein inhibit formation of the scrapie isoform [published erratum appears in J Cell Biol 1995 Jul;130(2):501] (1995) (433)
- Structural studies of the scrapie prion protein by electron crystallography (2002) (421)
- Prion biology and diseases. (1999) (420)
- Linkage of prion protein and scrapie incubation time genes (1986) (399)
- Pathway Complexity of Prion Protein Assembly into Amyloid* (2002) (399)
- Biology and genetics of prions causing neurodegeneration. (2013) (380)
- Scrapie-infected murine neuroblastoma cells produce protease-resistant prion proteins (1988) (375)
- Binding of neural cell adhesion molecules (N-CAMs) to the cellular prion protein. (2001) (370)
- Characterization of cloned cDNA representing rat myelin basic protein: Absence of expression in brain of shiverer mutant mice (1983) (365)
- Transmission of multiple system atrophy prions to transgenic mice (2013) (361)
- Molecular cloning of a human prion protein cDNA. (1986) (351)
- Measurement of the scrapie agent using an incubation time interval assay (1982) (346)
- Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins (1994) (344)
- A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform. (1997) (343)
- Purified and synthetic Alzheimer’s amyloid beta (Aβ) prions (2012) (339)
- Regional mapping of prion proteins in brain. (1992) (328)
- Purification and properties of the cellular and scrapie hamster prion proteins. (1988) (326)
- Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes (1993) (326)
- Analysis of proteome dynamics in the mouse brain (2010) (322)
- Transmissible and genetic prion diseases share a common pathway of neurodegeneration (1999) (317)
- Predicted alpha-helical regions of the prion protein when synthesized as peptides form amyloid. (1992) (314)
- Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein. (1994) (311)
- Identification of the Cu2+ binding sites in the N-terminal domain of the prion protein by EPR and CD spectroscopy. (2000) (310)
- Slow transmissible diseases of the nervous system (1979) (305)
- INSERTION IN PRION PROTEIN GENE IN FAMILIAL CREUTZFELDT-IAKOB DISEASE (1989) (301)
- A hypothalamic neuronal cell line persistently infected with scrapie prions exhibits apoptosis (1997) (298)
- Diversity of oligosaccharide structures linked to asparagines of the scrapie prion protein. (1989) (295)
- Scrapie prion proteins accumulate in the cytoplasm of persistently infected cultured cells (1990) (289)
- Chemical chaperones interfere with the formation of scrapie prion protein. (1996) (287)
- Perturbation of the secondary structure of the scrapie prion protein under conditions that alter infectivity. (1993) (287)
- Synthesis and trafficking of prion proteins in cultured cells. (1992) (280)
- Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein. (1994) (278)
- Diagnosis of human prion disease. (2005) (277)
- Glycosylinositol phospholipid anchors of the scrapie and cellular prion proteins contain sialic acid. (1992) (269)
- Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease. (1991) (266)
- Glycosylation differences between the normal and pathogenic prion protein isoforms. (1999) (265)
- Molecular biology and pathogenesis of prion diseases. (1996) (265)
- Serial propagation of distinct strains of Aβ prions from Alzheimer’s disease patients (2014) (263)
- Homozygosity for prion protein alleles encoding glutamine-171 renders sheep susceptible to natural scrapie. (1994) (260)
- Copper coordination in the full-length, recombinant prion protein. (2003) (259)
- Differential release of cellular and scrapie prion proteins from cellular membranes by phosphatidylinositol-specific phospholipase C. (1990) (258)
- Elimination of prions by branched polyamines and implications for therapeutics. (1999) (257)
- Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice. (1996) (257)
- Antibodies to a scrapie prion protein (1984) (256)
- Scrapie prion rod formation in vitro requires both detergent extraction and limited proteolysis (1991) (255)
- Human prion diseases (1994) (254)
- Is Parkinson's disease a prion disorder? (2009) (252)
- Changes in the localization of brain prion proteins during scrapie infection (1987) (248)
- Strain‐specified relative conformational stability of the scrapie prion protein (2001) (244)
- Measuring prions causing bovine spongiform encephalopathy or chronic wasting disease by immunoassays and transgenic mice (2002) (244)
- Chimeric prion protein expression in cultured cells and transgenic mice (1992) (243)
- Nerve growth factor increases mRNA levels for the prion protein and the beta-amyloid protein precursor in developing hamster brain. (1988) (239)
- Cholesterol Depletion and Modification of COOH-Terminal Targeting Sequence of the Priori Protein Inhibit Formation of the Scrapie Isoform (2002) (238)
- Mutant prion proteins in Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles (1992) (237)
- Small-molecule aggregates inhibit amyloid polymerization. (2008) (234)
- Evidence for assembly of prions with left-handed beta-helices into trimers. (2004) (232)
- Trafficking of prion proteins through a caveolae-mediated endosomal pathway (2003) (229)
- Copper binding to octarepeat peptides of the prion protein monitored by mass spectrometry (2008) (229)
- A Change in the Conformation of Prions Accompanies the Emergence of a New Prion Strain (2002) (224)
- Mice deficient for prion protein exhibit normal neuronal excitability and synaptic transmission in the hippocampus. (1996) (222)
- The Priori Diseases (1998) (221)
- Prion detection by an amyloid seeding assay (2007) (220)
- Cultured Cell Sublines Highly Susceptible to Prion Infection (2000) (220)
- Scrapie agent contains a hydrophobic protein. (1981) (219)
- Cytosolic Prion Protein in Neurons (2003) (217)
- A mouse prion protein transgene rescues mice deficient for the prion protein gene from purkinje cell degeneration and demyelination. (1999) (215)
- Branched Polyamines Cure Prion-Infected Neuroblastoma Cells (2001) (215)
- Continuum of prion protein structures enciphers a multitude of prion isolate-specified phenotypes (2006) (214)
- Prion diseases of humans and animals (1996) (214)
- The enzymes of glutamine metabolism (1973) (212)
- COOH-terminal sequence of the cellular prion protein directs subcellular trafficking and controls conversion into the scrapie isoform. (1997) (212)
- Ultrastructural localization of scrapie prion proteins in cytoplasmic vesicles of infected cultured cells. (1991) (212)
- Molecular properties, partial purification, and assay by incubation period measurements of the hamster scrapie agent. (1980) (211)
- Scrapie PrP 27-30 is a sialoglycoprotein (1985) (210)
- Cloning and characterization of the myelin basic protein gene from mouse: one gene can encode both 14 kd and 18.5 kd MBPs by alternate use of exons (1985) (210)
- Design and construction of diverse mammalian prion strains (2009) (207)
- Selective Neuronal Targeting in Prion Disease (1997) (206)
- Mapping the Prion Protein Using Recombinant Antibodies (1998) (205)
- Prion Protein of 106 Residues Creates an Artificial Transmission Barrier for Prion Replication in Transgenic Mice (1999) (202)
- Some speculations about prions, amyloid, and Alzheimer's disease. (1984) (202)
- Solution structure of Syrian hamster prion protein rPrP(90-231). (1999) (201)
- Complete genomic sequence and analysis of the prion protein gene region from three mammalian species. (1998) (201)
- Replication of distinct scrapie prion isolates is region specific in brains of transgenic mice and hamsters. (1992) (200)
- Immunoaffinity purification and neutralization of scrapie prion infectivity. (1988) (200)
- Linkage of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease to the prion protein gene (1992) (199)
- Etiology and pathogenesis of prion diseases. (1995) (197)
- Natural and synthetic prion structure from X-ray fiber diffraction (2009) (193)
- Prion protein gene variation among primates. (1997) (193)
- High-level expression and characterization of a purified 142-residue polypeptide of the prion protein. (1996) (192)
- Acquisition of protease resistance by prion proteins in scrapie-infected cells does not require asparagine-linked glycosylation. (1990) (190)
- Strain-specified characteristics of mouse synthetic prions. (2005) (188)
- Genetic and infectious prion diseases. (1993) (188)
- Propagation of prions causing synucleinopathies in cultured cells (2015) (188)
- Inherited prion diseases. (1994) (187)
- Folding of Prion Protein to Its Native α-Helical Conformation Is under Kinetic Control* (2001) (187)
- Prion protein peptides induce alpha-helix to beta-sheet conformational transitions. (1995) (186)
- Purification and properties of the cellular prion protein from Syrian hamster brain (1992) (185)
- Proposed three-dimensional structure for the cellular prion protein. (1994) (184)
- Recombinant prion protein induces rapid polarization and development of synapses in embryonic rat hippocampal neurons in vitro (2005) (183)
- Recombinant scrapie-like prion protein of 106 amino acids is soluble. (1996) (182)
- 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. (1991) (182)
- Amyloid plaques in Creutzfeldt‐Jakob disease stain with prion protein antibodies (1986) (180)
- Conversion of truncated and elongated prion proteins into the scrapie isoform in cultured cells. (1993) (180)
- Transmission of Elk and Deer Prions to Transgenic Mice (2006) (180)
- Doxycycline control of prion protein transgene expression modulates prion disease in mice. (1998) (179)
- Purified prion proteins and scrapie infectivity copartition into liposomes. (1987) (179)
- The prion diseases. (1998) (178)
- Prion protein conformation in a patient with sporadic fatal insomnia. (1999) (173)
- Conversion of a-helices into ,f-sheets features in the formation of the scrapie prion proteins (2005) (172)
- Prion diseases and neurodegeneration. (1994) (170)
- Identification of glycoinositol phospholipid linked and truncated forms of the scrapie prion protein. (1990) (169)
- Biology and genetics of prion diseases. (1994) (168)
- Inherited human prion diseases (1990) (168)
- Two different neurodegenerative diseases caused by proteins with similar structures (2001) (168)
- Proteinase‐resistant prion protein accumulation in Syrian hamster brain correlates with regional pathology and scrapie infectivity (1991) (168)
- Conformational Transformations in Peptides Containing Two Putative α-Helices of the Prion Protein (1995) (167)
- Doppel is an N-glycosylated, glycosylphosphatidylinositol-anchored protein. Expression in testis and ectopic production in the brains of Prnp(0/0) mice predisposed to Purkinje cell loss. (2000) (165)
- Mimicking dominant negative inhibition of prion replication through structure-based drug design. (2000) (164)
- Time-controlled transcardiac perfusion cross-linking for the study of protein interactions in complex tissues (2004) (164)
- Asymptomatic deer excrete infectious prions in faeces (2010) (164)
- Identification of a prion protein epitope modulating transmission of bovine spongiform encephalopathy prions to transgenic mice. (1997) (164)
- Local structural plasticity of the prion protein. Analysis of NMR relaxation dynamics. (2001) (163)
- Immunolocalization of heparan sulfate proteoglycans to the prion protein amyloid plaques of Gerstmann-Straussler syndrome, Creutzfeldt-Jakob disease and scrapie. (1990) (163)
- Prion isolate specified allotypic interactions between the cellular and scrapie prion proteins in congenic and transgenic mice. (1994) (160)
- Distinct synthetic Aβ prion strains producing different amyloid deposits in bigenic mice (2014) (160)
- Genetics and polymorphism of the mouse prion gene complex: control of scrapie incubation time (1988) (159)
- X-ray diffraction of scrapie prion rods and PrP peptides. (1995) (159)
- Protease-Sensitive Synthetic Prions (2010) (159)
- A prion protein variant in a family with the telencephalic form of Gerstmann‐Sträussler‐Scheinker syndrome (1991) (159)
- Prion protein gene variation among primates. (1995) (157)
- Prions in skeletal muscle (2002) (155)
- Dominant-negative inhibition of prion replication in transgenic mice (2002) (155)
- Molecular characteristics of the major scrapie prion protein. (1984) (155)
- Genetics of prions. (1997) (153)
- Doppel-induced cerebellar degeneration in transgenic mice (2001) (152)
- Physical studies of conformational plasticity in a recombinant prion protein. (1997) (149)
- Potent inhibition of scrapie prion replication in cultured cells by bis-acridines (2003) (149)
- Huntington disease phenocopy is a familial prion disease. (2001) (149)
- Induction of Distinct [URE3] Yeast Prion Strains (2001) (149)
- Transmission and detection of prions in feces. (2008) (147)
- The prion folding problem. (1997) (146)
- Locally disordered conformer of the hamster prion protein: a crucial intermediate to PrPSc? (2002) (144)
- Viroids and prions. (1982) (143)
- Prion-protein immunoreactivity in human transmissible dementias. (1986) (142)
- Mechanisms of prion protein assembly into amyloid (2008) (142)
- Prion glycoprotein: structure, dynamics, and roles for the sugars. (2001) (141)
- Mutant PrPSc Conformers Induced by a Synthetic Peptide and Several Prion Strains (2004) (137)
- Copper-catalyzed oxidation of the recombinant SHa(29–231) prion protein (2001) (137)
- Non-hydrophobic extracytoplasmic determinant of stop transfer in the prion protein (1990) (136)
- Release of the cellular prion protein from cultured cells after loss of its glycoinositol phospholipid anchor. (1993) (136)
- Unusual topogenic sequence directs prion protein biogenesis. (1990) (135)
- Prions and neurodegenerative diseases. (1987) (135)
- Molecular cloning of a candidate chicken prion protein. (1992) (134)
- Search for a Prion-Specific Nucleic Acid (2005) (132)
- Scrapie prion liposomes and rods exhibit target sizes of 55,000 Da. (1988) (132)
- Abbreviated incubation times for human prions in mice expressing a chimeric mouse–human prion protein transgene (2003) (130)
- Attempts to restore scrapie prion infectivity after exposure to protein denaturants. (1993) (129)
- Further analysis of nucleic acids in purified scrapie prion preparations by improved return refocusing gel electrophoresis. (1992) (129)
- A rapid radioactive assay for glutamine synthetase, glutaminase, asparagine synthetase, and asparaginase. (1970) (128)
- Continuous Quinacrine Treatment Results in the Formation of Drug-Resistant Prions (2009) (128)
- Quantitative trait loci affecting prion incubation time in mice. (2000) (128)
- Neuropeptide Y: some viewpoints on a multifaceted peptide in the normal and diseased nervous system 1 Published on the World Wide Web on 4 December 1997. 1 (1998) (128)
- Identification of Candidate Proteins Binding to Prion Protein (1997) (126)
- Creutzfeldt-Jakob disease prion proteins in human brains. (1985) (126)
- Scrapie prions. (1989) (126)
- Heritable disorder resembling neuronal storage disease in mice expressing prion protein with deletion of an α-helix (1997) (125)
- Monoclonal antibodies to the cellular and scrapie prion proteins. (1986) (125)
- RAPID DETECTION OF CREUTZFELDT-JAKOB DISEASE AND SCRAPIE PRION PROTEINS: 88 (1990) (125)
- Tau prions from Alzheimer’s disease and chronic traumatic encephalopathy patients propagate in cultured cells (2016) (125)
- A synthetic peptide initiates Gerstmann-Sträussler-Scheinker (GSS) disease in transgenic mice. (2000) (122)
- Biogenesis and Transmembrane Orientation of the Cellular Isoform of the Scrapie Prion Protein (1987) (120)
- Oxidative metabolism in cells isolated from brown adipose tissue. 1. Catecholamine and fatty acid stimulation of respiration. (1968) (119)
- Paradoxical shortening of scrapie incubation times by expression of prion protein transgenes derived from long incubation period mice (1991) (119)
- Prions and prion proteins 1 (1991) (117)
- 2-Aminothiazoles as therapeutic leads for prion diseases. (2011) (116)
- Circumventing tolerance to generate autologous monoclonal antibodies to the prion protein. (1996) (116)
- Discovery of 2-Aminothiazoles as Potent Antiprion Compounds (2009) (114)
- Prion clearance in bigenic mice. (2005) (114)
- Drug resistance confounding prion therapeutics (2013) (114)
- Genes contributing to prion pathogenesis. (2008) (114)
- Asymptomatic deer excrete infectious prions in feces (2009) (113)
- A Data Processing Pipeline for Mammalian Proteome Dynamics Studies Using Stable Isotope Metabolic Labeling* (2011) (113)
- Search for a putative scrapie genome in purified prion fractions reveals a paucity of nucleic acids. (1991) (111)
- Quinacrine treatment trial for sporadic Creutzfeldt-Jakob disease (2013) (110)
- Structure and polymorphism of the mouse prion protein gene. (1994) (110)
- Genetic control of scrapie and Creutzfeldt-Jakob disease in mice. (1983) (109)
- Prion protein (PrP) synthetic peptides induce cellular PrP to acquire properties of the scrapie isoform. (1995) (109)
- Prion Uptake in the Gut: Identification of the First Uptake and Replication Sites (2011) (109)
- Rapid detection of Creutzfeldt‐Jakob disease and scrapie prion proteins (1990) (108)
- Inactivation of Prions by Acidic Sodium Dodecyl Sulfate (2006) (107)
- Epitope mapping of the Syrian hamster prion protein utilizing chimeric and mutant genes in a vaccinia virus expression system. (1991) (106)
- Three scrapie prion isolates exhibit different accumulation patterns of the prion protein scrapie isoform. (1993) (105)
- Mutation and polymorphism of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease (CJD). (1993) (104)
- Thiocyanate and hydroxyl ions inactivate the scrapie agent. (1981) (103)
- A microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions (1985) (102)
- Purified scrapie prions resist inactivation by UV irradiation (1987) (101)
- Rapid Acquisition of β-Sheet Structure in the Prion Protein Prior to Multimer Formation (1998) (101)
- Disruption of prion rods generates 10-nm spherical particles having high alpha-helical content and lacking scrapie infectivity (1996) (100)
- Insoluble wild–type and protease–resistant mutant prion protein in brains of patients with inherited prion disease (1996) (100)
- Transmission Barriers for Bovine, Ovine, and Human Prions in Transgenic Mice (2005) (99)
- Directed evolution of an anti-prion protein scFv fragment to an affinity of 1 pM and its structural interpretation. (2006) (99)
- Separation of scrapie prion infectivity from PrP amyloid polymers. (1996) (99)
- Chemistry and biology of prions. (1992) (99)
- Identification of cellular proteins binding to the scrapie prion protein. (1990) (98)
- Scrapie prions selectively modify the stress response in neuroblastoma cells. (1995) (98)
- Identification of Two Prion Protein Regions That Modify Scrapie Incubation Time (2001) (98)
- Bioluminescence imaging of Aβ deposition in bigenic mouse models of Alzheimer's disease (2011) (97)
- Evidence That Bank Vole PrP Is a Universal Acceptor for Prions (2014) (96)
- Structure-activity relationship study of prion inhibition by 2-aminopyridine-3,5-dicarbonitrile-based compounds: parallel synthesis, bioactivity, and in vitro pharmacokinetics. (2007) (94)
- Structural heterogeneity and intersubject variability of Aβ in familial and sporadic Alzheimer’s disease (2018) (92)
- Immunologic and molecular biologic studies of prion proteins in bovine spongiform encephalopathy. (1993) (92)
- Prions are novel infectious pathogens causing scrapie and Creutzfeldt-Jakob disease. (1986) (91)
- Partial purification and evidence for multiple molecular forms of the scrapie agent. (1978) (91)
- Propagation of prion strains through specific conformers of the prion protein (1997) (90)
- Scrapie prions alter receptor‐mediated calcium responses in cultured cells (1993) (90)
- Cryo-Immunogold Electron Microscopy for Prions: Toward Identification of a Conversion Site (2008) (89)
- Evidence for a secretory form of the cellular prion protein. (1987) (89)
- Three hamster species with different scrapie incubation times and neuropathological features encode distinct prion proteins (1990) (89)
- Sulfated glycosaminoglycans in amyloid plaques of prion diseases (2004) (88)
- Resistance of Bovine Spongiform Encephalopathy (BSE) Prions to Inactivation (2008) (88)
- Dominant-Negative Inhibition of Prion Formation Diminished by Deletion Mutagenesis of the Prion Protein (2000) (88)
- Notch-1 activation and dendritic atrophy in prion disease. (2005) (88)
- Thermodynamics of model prions and its implications for the problem of prion protein folding. (1999) (87)
- Oxidative metabolism in cells isolated from brown adipose tissue. 2. Catecholamine regulated respiratory control. (1968) (86)
- Structural Intermediates in the Putative Pathway from the Cellular Prion Protein to the Pathogenic Form (2001) (86)
- Conformational propagation with prion‐like characteristics in a simple model of protein folding (2001) (86)
- Predicted secondary structure and membrane topology of the scrapie prion protein. (1987) (86)
- Cell division modulates prion accumulation in cultured cells (2007) (85)
- Conservation of the cellular gene encoding the scrapie prion protein. (1986) (84)
- 1 An Introduction to Prion Biology and Diseases (2004) (83)
- Transmission of scrapie in hamsters. (1985) (83)
- Complete Penetrance of Creutzfeldt-Jakob Disease in Libyan Jews Carrying the E200K Mutation in the Prion Protein Gene (1995) (82)
- Immunoblotting of Creutzfeldt‐Jakob disease prion proteins: Host species—specific epitopes (1987) (82)
- Intracellular accumulation of the cellular prion protein after mutagenesis of its Asn-linked glycosylation sites. (1990) (81)
- Selective precipitation of prions by polyoxometalate complexes. (2005) (80)
- Regulation of glutamine synthetase from Bacillus subtilis by divalent cations, feedback inhibitors, and L-glutamine. (1974) (79)
- Purified scrapie prions resist inactivation by procedures that hydrolyze, modify, or shear nucleic acids. (1987) (79)
- De novo generation of prion strains (2011) (78)
- Mouse Models for Studying the Formation and Propagation of Prions* (2014) (77)
- Reversible chemical modification of the scrapie agent. (1981) (77)
- Aβ and tau prion-like activities decline with longevity in the Alzheimer’s disease human brain (2019) (77)
- CNS amyloid proteins in neurodegenerative diseases (1988) (77)
- Decreased receptor-mediated calcium response in prion-infected cells correlates with decreased membrane fluidity and IP sub 3 release (1996) (75)
- Oxidation of methionine residues in the prion protein by hydrogen peroxide. (2004) (75)
- Antibodies to the scrapie protein decorate prion rods. (1985) (75)
- Colloid Formation by Drugs in Simulated Intestinal Fluid (2010) (74)
- Prion Protein Isoforms, a Convergence of Biological and Structural Investigations (*) (1995) (73)
- Solid‐state NMR studies of the prion protein H1 fragment (1996) (73)
- Molecular biology and genetics of prion diseases. (1994) (73)
- The peculiar nature of unfolding of the human prion protein (2004) (72)
- Expression of unglycosylated mutated prion protein facilitates PrP(Sc) formation in neuroblastoma cells infected with different prion strains. (2000) (72)
- Developmental expression of PrP in the post-implantation embryo (2007) (72)
- Scrapie and Creutzfeldt-Jakob disease prion proteins share physical properties and antigenic determinants. (1985) (72)
- Folding of prion protein to its native alpha-helical conformation is under kinetic control. (2001) (71)
- Solid-state NMR studies of the secondary structure of a mutant prion protein fragment of 55 residues that induces neurodegeneration (2001) (71)
- Prion disease (PrP‐A117V) presenting with ataxia instead of dementia (1995) (71)
- Scrapie and cellular prion proteins share polypeptide epitopes. (1986) (70)
- Differences between the prion protein and its homolog Doppel: a partially structured state with implications for scrapie formation. (2002) (70)
- Primary structure of prion protein may modify scrapie isolate properties. (1989) (70)
- Structural Polymorphism of Alzheimer's β-Amyloid Fibrils as Controlled by an E22 Switch: A Solid-State NMR Study. (2016) (69)
- Prion protein mutation at codon 102 in an Italian family with Gerstmann‐Sträussler‐Scheinker syndrome (1992) (69)
- RESISTANCE OF THE SCRAPIE AGENT TO INACTIVATION BY PSORALENS (1983) (68)
- DNA length polymorphism 5′ to the myelin basic protein gene is associated with multiple sclerosis (1990) (68)
- Familial Creutzfeldt-Jakob disease. Codon 200 prion disease in Libyan Jews. (1997) (67)
- Assembly of natural and recombinant prion protein into fibrils (2005) (67)
- Spontaneous generation of rapidly transmissible prions in transgenic mice expressing wild-type bank vole prion protein (2012) (66)
- MSA prions exhibit remarkable stability and resistance to inactivation (2017) (66)
- Prions: novel infectious pathogens. (1984) (66)
- Adenosine 3':5'-cyclic monophosphate control of the enzymes of glutamine metabolism in Escherichia coli. (1972) (66)
- Spontaneous generation of anchorless prions in transgenic mice (2011) (64)
- Self-assembly of recombinant prion protein of 106 residues. (2000) (64)
- Compartment modeling for mammalian protein turnover studies by stable isotope metabolic labeling. (2012) (63)
- Perspectives on prion biology, prion disease pathogenesis, and pharmacologic approaches to treatment. (2003) (62)
- Search for a scrapie-specific nucleic acid: a progress report. (1988) (62)
- α-Synuclein: Multiple System Atrophy Prions. (2018) (60)
- Sedimentation characteristics of the scrapie agent from murine spleen and brain. (1978) (60)
- Immobilized prion protein undergoes spontaneous rearrangement to a conformation having features in common with the infectious form (2001) (60)
- Experimental scrapie in mice: Ultrastructural observations (1978) (60)
- Prion infection of mouse neurospheres. (2006) (59)
- Permethylation and tandem mass spectrometry of oligosaccharides having free hexosamine: analysis of the glycoinositol phospholipid anchor glycan from the scrapie prion protein. (1990) (59)
- Properties of scrapie prion protein liposomes. (1988) (59)
- Kuru with incubation periods exceeding two decades (1982) (59)
- Culture and Characterization of Epithelial Cells from Bovine Choroid Plexus (1981) (58)
- Kinetics and mechanism of amyloid formation by the prion protein H1 peptide as determined by time-dependent ESR. (1997) (58)
- Tau aggregates are RNA-protein assemblies that mislocalize multiple nuclear speckle components (2021) (57)
- Mutation of the prion protein gene at codon 208 in familial Creutzfeldt-Jakob disease (1996) (57)
- HIV-1 : Fifteen Proteins and an RNA (2006) (57)
- Repetitive DNA (TGGA)n 5' to the human myelin basic protein gene: a new form of oligonucleotide repetitive sequence showing length polymorphism. (1990) (57)
- Molecular characteristics of prion rods purified from scrapie-infected hamster brains. (1986) (56)
- The Neurodegeneration Sequence in Prion Diseases: Evidence from Functional, Morphological and Ultrastructural Studies of the GABAergic System (2004) (56)
- β-Amyloid Prions and the Pathobiology of Alzheimer's Disease. (2018) (56)
- Electrophoretic properties of the scrapie agent in agarose gels. (1980) (56)
- Salivary prions in sheep and deer (2012) (56)
- The prion domain of yeast Ure2P induces autocatalytic formation of amyloid fibers by a recombinant fusion protein (2008) (55)
- Prion proteins with pathogenic and protective mutations show similar structure and dynamics. (2009) (55)
- Quantitative traits of prion strains are enciphered in the conformation of the prion protein. (2000) (55)
- Progressive retinal degeneration in scrapie-infected hamsters: a light and electron microscopic analysis. (1981) (55)
- Scrapie in mice deficient in apolipoprotein E or glial fibrillary acidic protein (1996) (55)
- Measuring prions by bioluminescence imaging (2009) (55)
- Antibody binding defines a structure for an epitope that participates in the PrPC-->PrPSc conformational change. (1999) (53)
- Multiple system atrophy prions retain strain specificity after serial propagation in two different Tg(SNCA*A53T) mouse lines (2019) (53)
- Cryptic epitopes in N‐terminally truncated prion protein are exposed in the full‐length molecule: Dependence of conformation on pH (2001) (52)
- Thioaptamer interactions with prion proteins: sequence-specific and non-specific binding sites. (2007) (51)
- The Neurochemistry of Prion Diseases (1993) (51)
- Molecular properties of complexes formed between the prion protein and synthetic peptides. (1997) (51)
- A 31-residue peptide induces aggregation of tau's microtubule-binding region in cells. (2017) (50)
- Developmental expression of prion protein gene in brain. (1987) (49)
- Lipid Composition in Scrapie‐Infected Mouse Brain: Prion Infection Increases the Levels of Dolichyl Phosphate and Ubiquinone (1996) (49)
- Bitemporal hypometabolism in Creutzfeldt-Jakob disease measured by positron emission tomography with [18F]-2-fluorodeoxyglucose. (1984) (49)
- Prion protein gene expression in cultured cells. (1988) (48)
- Differential Inhibition of Prion Propagation by Enantiomers of Quinacrine (2003) (48)
- Structures of prion proteins and conformational models for prion diseases. (1996) (48)
- Human prion strain selection in transgenic mice (2010) (47)
- Immuno-gold localization of prion filaments in scrapie-infected hamster brains. (1987) (47)
- Conformational transformation and selection of synthetic prion strains. (2011) (47)
- Inherited prion disease caused by the V210I mutation (2001) (47)
- Unraveling prion diseases through molecular genetics (1989) (47)
- Prion protein preamyloid and amyloid deposits in Gerstmann-Sträussler-Scheinker disease, Indiana kindred. (1992) (47)
- Human prions and plasma lipoproteins. (2006) (47)
- Thermodynamic Considerations of Mammalian Thermogenesis (1968) (47)
- Sedimentation properties of the scrapie agent. (1977) (46)
- Prions causing degenerative neurological diseases. (1987) (46)
- The PrP-like Protein Doppel Binds Copper* (2003) (46)
- A Protease-Resistant 61-Residue Prion Peptide Causes Neurodegeneration in Transgenic Mice (2001) (46)
- Attempts to convert the cellular prion protein into the scrapie isoform in cell-free systems (1992) (45)
- A γ-secretase inhibitor and quinacrine reduce prions and prevent dendritic degeneration in murine brains (2008) (45)
- Protease-Resistant Prions Selectively Decrease Shadoo Protein (2011) (45)
- Expression and targeting of Syrian hamster prion protein induced by heat shock in transgenic Drosophila melanogaster (1995) (44)
- Solid-state NMR structural studies of the fibril form of a mutant mouse prion peptide PrP89-143(P101L). (2006) (44)
- Immunoglobulins in Urine of Hamsters with Scrapie* (2004) (44)
- Brain tissue from persons dying of creutzfeldt‐jakob disease causes scrapie‐like encephalopathy in goats (1980) (44)
- Molecular biology and transgenetics of prion diseases. (1991) (43)
- QUINACRINE IS MAINLY METABOLIZED TO MONO-DESETHYL QUINACRINE BY CYP3A4/5 AND ITS BRAIN ACCUMULATION IS LIMITED BY P-GLYCOPROTEIN (2006) (43)
- Presence of prion protein in peripheral tissues of Libyan Jews with Creutzfeldt‐Jakob disease (1992) (43)
- Characterization of prion proteins with monospecific antisera to synthetic peptides. (1988) (42)
- Gel electrophoresis and glass permeation chromatography of the hamster scrapie agent after enzymatic digestion and detergent extraction. (1980) (42)
- Conformational transitions in peptides containing two putative alpha-helices of the prion protein. (1995) (42)
- Prion protein expression in Chinese hamster ovary cells using a glutamine synthetase selection and amplification system. (1997) (42)
- Host-to-Parasite Gene Transfer in Flowering Plants: Phylogenetic Evidence from Malpighiales (2004) (42)
- Prion protein structure and scrapie replication: theoretical, spectroscopic, and genetic investigations. (1996) (42)
- Ultrastructural studies on scrapie prion protein crystals obtained from reverse micellar solutions. (1999) (42)
- Discovering the Cause of AIDS (2002) (42)
- Cooperative binding of dominant-negative prion protein to kringle domains. (2003) (42)
- Developing Therapeutics for PrP Prion Diseases. (2017) (41)
- Prion Protein Transgenes and the Neuropathology in Prion Diseases (1995) (41)
- Human prion diseases and neurodegeneration. (1996) (40)
- Intracerebral Infusion of Antisense Oligonucleotides Into Prion-infected Mice (2012) (40)
- Retinal degeneration in experimental Creutzfeldt-Jakob disease. (1983) (40)
- Changes in the localization of brain prion proteins during scrapie infection (1998) (40)
- Development of antibody fragments for immunotherapy of prion diseases. (2009) (40)
- Transmission of scrapie and sheep-passaged bovine spongiform encephalopathy prions to transgenic mice expressing elk prion protein. (2009) (40)
- Nucleic acids in prion preparations: unspecific background or essential component? (1994) (40)
- Conformation of PrP(C) on the cell surface as probed by antibodies. (2003) (39)
- Scrapie infection of transgenic mice leads to network and intrinsic dysfunction of cortical and hippocampal neurones (1994) (39)
- Scrapie‐Associated Particles in Postsynaptic Processes Further Ultrastructural Studies (1981) (39)
- Scrapie Infection Diminishes Spines and Increases Varicosities of Dendrites in Hamsters: A Quantitative Golgi Analysis (1987) (39)
- CREUTZFELDT‐JAKOB DISEASE AND SCRAPIE PRIONS (1989) (39)
- Different 2-Aminothiazole Therapeutics Produce Distinct Patterns of Scrapie Prion Neuropathology in Mouse Brains (2015) (38)
- Replication of scrapie prions in hamster eyes precedes retinal degeneration. (1986) (38)
- Pharmacokinetics of quinacrine in the treatment of prion disease (2004) (38)
- Prion protein genes: evolutionary and functional aspects. (1991) (37)
- On the biology of prions (2004) (37)
- Regulation of glutaminase B in Escherichia coli. I. Purification, properties, and cold lability. (1976) (36)
- Electron crystallography of the scrapie prion protein complexed with heavy metals. (2007) (36)
- Delimiting the location of the scrapie prion incubation time gene on chromosome 2 of the mouse. (1993) (36)
- Structural changes in a hydrophobic domain of the prion protein induced by hydration and by ala-->Val and pro-->Leu substitutions. (2000) (36)
- Towards authentic transgenic mouse models of heritable PrP prion diseases (2016) (35)
- Hormones and Neurotransmitters Control Cyclic AMP Metabolism in Choroid Plexus Epithelial Cells (1984) (35)
- Molecular Biology and Pathology of Scrapie and the Prion Diseases of Humans (1991) (35)
- Scrapie infectivity is independent of amyloid staining properties of the N-terminally truncated prion protein. (2000) (34)
- Immunological studies of scrapie infection (1982) (34)
- Convergent replication of mouse synthetic prion strains. (2013) (34)
- Determinants of carboxyl-terminal domain translocation during prion protein biogenesis. (1994) (34)
- Prion protein preamyloid and amyloid deposits in Gerstmann-Sträussler-Scheinker disease, Indiana kindred (1992) (33)
- Theoretical studies of sequence effects on the conformational properties of a fragment of the prion protein: implications for scrapie formation. (1995) (33)
- Biaryl Amides and Hydrazones as Therapeutics for Prion Disease in Transgenic Mice (2013) (33)
- Prions: so many fibers, so little infectivity. (2004) (32)
- Prions causing nervous system degeneration. (1987) (32)
- Prion Protein-Antibody Complexes Characterized by Chromatography-Coupled Small-Angle X-Ray Scattering. (2015) (32)
- Kinetics of prion protein accumulation in the CNS of mice with experimental scrapie. (1999) (32)
- Spectroscopic Characterization of Conformational Differences between PrP $^{\text{C}}$ and PrP $^{\text{Sc}}$ : An $\alpha $ -helix to $\beta $ -sheet Transition (1994) (32)
- Structure-activity relationship study of 9-aminoacridine compounds in scrapie-infected neuroblastoma cells. (2006) (32)
- 11 Scrapie, Chronic Wasting Disease, and Transmissible Mink Encephalopathy (2004) (31)
- Molecular studies of prion diseases. (1998) (31)
- Familial Parkinson’s point mutation abolishes multiple system atrophy prion replication (2017) (31)
- Expression mapping of tetracycline-responsive prion protein promoter: Digital atlasing for generating cell-specific disease models (2006) (31)
- Experimental Scrapie in the Mouse: Electrophoretic and Sedimentation Properties of the Partially Purified Agent (1980) (31)
- Analysis of nucleic acids in purified scrapie prion preparations. (1993) (31)
- 2 Development of the Prion Concept (1999) (30)
- Prion and doppel proteins bind to granule cells of the cerebellum (2002) (30)
- Marked decrease of neuropeptide Y Y2 receptor binding sites in the hippocampus in murine prion disease. (1997) (30)
- Surface charge of polyoxometalates modulates polymerization of the scrapie prion protein (2009) (30)
- Prions: Methods for Assay, Purification, and Characterization (1984) (29)
- Phosphorothioate Oligonucleotides Reduce PrPSc Levels and Prion Infectivity in Cultured Cells (2007) (29)
- Prions Prions Prions (1996) (29)
- Transgenic models of prion disease. (2000) (29)
- Tandem mass spectrometry of peptides with N-terminal glutamine studies on a prion protein peptide (1990) (29)
- Transgenetic investigations of prion diseases of humans and animals. (1993) (29)
- Erratum: Prion protein preamyloid and amyloid deposits in Gerstmann- Straussler-Scheinker disease, Indiana kindred (Proc. Natl. Acad. Sci. USA (October 1, 1992) 89:19 (9349-9353)) (1993) (29)
- A novel mechanism for group translocation: Substrate‐product reutilization by γ‐glutamyl transpeptidase in peptide and amino acid transport (1976) (28)
- Prion proteins in subpopulations of white blood cells from patients with sporadic Creutzfeldt-Jakob disease (2009) (28)
- Electrospray mass spectrometry of the glycosylinositol phospholipid of the scrapie prion protein. (1991) (28)
- The space-time distribution of Hodgkin's disease in Connecticut, 1940-69. (1973) (27)
- Pharmacokinetics and Metabolism of 2-Aminothiazoles with Antiprion Activity in Mice (2013) (27)
- Early evidence that a protease-resistant protein is an active component of the infectious prion (2004) (27)
- Bioassays and Inactivation of Prions. (2017) (27)
- Replication of the Scrapie Agent in Hamster Brain Precedes Neuronal Vacuolation (1983) (27)
- Prions and nucleic acids: search for "residual" nucleic acids and screening for mutations in the PrP-gene. (1993) (27)
- Toward development of assays for scrapie-specific antibodies. (1981) (27)
- Prion encephalopathies of animals and humans. (1993) (26)
- Engineering the prion protein using chemical synthesis. (2001) (26)
- Pharmacokinetics of Quinacrine Efflux from Mouse Brain via the P-glycoprotein Efflux Transporter (2012) (26)
- Disorders of glutamate metabolism and neurological dysfunction. (1981) (26)
- Determination of scrapie agent titer from incubation period measurements in hamsters. (1981) (26)
- Novel compounds lowering the cellular isoform of the human prion protein in cultured human cells. (2014) (26)
- Prion diseases of the central nervous system. (1990) (26)
- Pyridine nucleotide changes during thermogenesis in brown fat tissue in vivo. (1968) (26)
- Plasma membrane invaginations containing clusters of full-length PrPSc are an early form of prion-associated neuropathology in vivo (2013) (26)
- Chemical Induction of Misfolded Prion Protein Conformers in Cell Culture* (2009) (25)
- PrPSc in scrapie-infected hamster brain is spatially and temporally related to histopathology and infectivity titer. (1989) (25)
- A Survey of Antiprion Compounds Reveals the Prevalence of Non-PrP Molecular Targets* (2011) (25)
- Structural studies of truncated forms of the prion protein PrP. (2015) (25)
- Degradation of fungal prion HET-s(218-289) induces formation of a generic amyloid fold. (2012) (25)
- The regulation of oxidative metabolism of isolated brown fat cells. (1968) (25)
- Experimental Models of Inherited PrP Prion Diseases. (2017) (25)
- Optimization of Aryl Amides that Extend Survival in Prion-Infected Mice (2016) (25)
- Prion liposomes. (1990) (25)
- Discovering DNA encodes heredity and prions are infectious proteins. (2006) (24)
- When sporadic disease is not sporadic: the potential for genetic etiology. (2004) (24)
- Acceleration of scrapie in neonatal Syrian hamsters (1989) (23)
- Molecular structure, biology, and genetics of prions. (1988) (23)
- Polio and nobel prizes: Looking back 50 years (2007) (23)
- N‐terminally tagged prion protein supports prion propagation in transgenic mice (1997) (23)
- Mechanism of Scrapie Prion Precipitation with Phosphotungstate Anions (2014) (23)
- Mutation in codon 200 and polymorphism in codon 129 of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease. (1994) (23)
- 3 Bioassays of Prions (1999) (23)
- Molecular biology of prions causing infectious and genetic encephalopathies of humans as well as scrapie of sheep and BSE of cattle. (1991) (22)
- MEASUREMENT OF INTERNUCLEAR DISTANCES BY SWITCHED ANGLE SPINNING NMR (1994) (22)
- 10 Cell Biology of Prions (1999) (22)
- Conformation-Dependent High-Affinity Monoclonal Antibodies to Prion Proteins (2010) (21)
- A high-throughput screening assay for determining cellular levels of total tau protein. (2013) (21)
- Detecting mad cow disease. (2004) (21)
- Natural and experimental prion diseases of humans and animals (1992) (21)
- Regulation and genetic control of brain amyloid (1991) (20)
- Kinetics of Human Mutant Tau Prion Formation in the Brains of 2 Transgenic Mouse Lines (2017) (20)
- Genetics of prion infections. (1991) (20)
- How an Infection of Sheep Revealed Prion Mechanisms in Alzheimer’s Disease and Other Neurodegenerative Disorders (2021) (20)
- Expanding spectrum of prion diseases. (2020) (20)
- Biology and structure of scrapie prions. (1986) (20)
- Separation of membrane-bound gamma-glutamyl transpeptidase from brush border transport and enzyme activities. (1980) (20)
- 2‐Aminothiazoles with Improved Pharmacotherapeutic Properties for Treatment of Prion Disease (2013) (20)
- Copper induces conformational changes in the N-terminal part of cell-surface PrPC (2006) (19)
- Molecular cloning of a mink prion protein gene. (1992) (19)
- Suppression of polyclonal B cell activation in scrapie-infected C3H/HeJ mice. (1978) (19)
- Evidence for sortilin modulating regional accumulation of human tau prions in transgenic mice (2017) (19)
- 9 Transgenetic Investigations of the Species Barrier and Prion Strains (2004) (19)
- Prion protein amyloid and neurodegeneration (1995) (19)
- Biohazards of investigations on the transmissible spongiform encephalopathies. (1980) (18)
- FoxO3 regulates neuronal reprogramming of cells from postnatal and aging mice (2016) (18)
- H2 Histamine Receptors on the Epithelial Cells of Choroid Plexus (1986) (18)
- Antiprion compounds that reduce PrP(Sc) levels in dividing and stationary-phase cells. (2013) (18)
- Discriminating between cellular and misfolded prion protein by using affinity to 9-aminoacridine compounds. (2007) (18)
- Modulation of Creutzfeldt‐Jakob disease prion propagation by the A224V mutation (2015) (18)
- Molecular biology and genetics of neurodegenerative diseases caused by prions. (1992) (18)
- RESEARCH ON SCRAPIE (1982) (18)
- SCRAPIE AND CELLULAR PRION PROTEINS DIFFER IN THEIR KINETICS OF SYNTHESIS AND TOPOLOGY IN CULTURED CELLS: 150 (1990) (17)
- Influence of Water, Fat, and Glycerol on the Mechanism of Thermal Prion Inactivation* (2007) (17)
- Use of a 2-aminothiazole to Treat Chronic Wasting Disease in Transgenic Mice. (2015) (17)
- Novel properties and biology of scrapie prions. (1991) (17)
- Spectroscopic evidence for the control of respiration prior to phosphorylation in hamster brown fat cells. (1970) (17)
- Selective neuronal vulnerability during experimental scrapie infection: Insights from an ultrastructural investigation (2000) (17)
- Spontaneous Neurodegeneration in Transgenic Mice with Prion Protein Codon 101 Proline → Leucine Substitution a (1991) (17)
- Different α-synuclein prion strains cause dementia with Lewy bodies and multiple system atrophy (2022) (16)
- Scanning for mutations in the human prion protein open reading frame by temporal temperature gradient gel electrophoresis (1995) (16)
- Scrapie prions, brain amyloid, and senile dementia. (1985) (16)
- Prions--infectious pathogens causing the spongiform encephalopathies. (1985) (16)
- Mitogen stimulation of splenocytes from mice infected with scrapie agent. (1978) (15)
- Application of rotational resonance to inhomogeneously broadened systems (1996) (15)
- PARTIAL PURIFICATION AND KINETICS OF γ‐GLUTAMYL TRANSPEPTIDASE FROM BOVINE CHOROID PLEXUS (1978) (15)
- Detecting prion protein gene mutations by denaturing gradient gel electrophoresis (1994) (15)
- A long‐lived Aβ oligomer resistant to fibrillization (2018) (15)
- Novel mechanisms of degeneration of the central nervous system--prion structure and biology. (1988) (15)
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- Affinity-Tagged Miniprion Derivatives Spontaneously Adopt Protease-Resistant Conformations (2000) (14)
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- Regulation of glutaminase B in Escherichia coli. II. Modulaltion of activity by carbosylate and borate ions. (1976) (14)
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- Regulation of glutaminase levels in Escherichia coli (1975) (13)
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- Doppel is an N-glycosylated, GPI-anchored protein: expression in testis and ectopic production in the brains of Prnp mice predisposed to Purkinje cell loss (2000) (13)
- Regulation of glutaminase B in Escherichia coli. III. Control by nucleotides and divalent cations. (1976) (13)
- Molecular Genetics and Transgenic Model of Gertsmann—Sträussler—Scheinker Disease (1991) (13)
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- Hypothermic Protection Against Cerebral Edema of Ischemia: Prevention of Cerebral Edema in the Rat After Prolonged Circulatory Arrest (1968) (12)
- Prion protein (PrP) is not involved in the pathogenesis of spongiform encephalopathy in zitter rats (1994) (12)
- Mass spectrometric analysis of a GPI-anchored protein: The scrapie prion protein (1993) (12)
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- Preservation by freezing of glucose and alanine transport into kidney membrane vesicles. (1978) (12)
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- Optimization of Arylamides as Novel, Potent and Brain-penetrant Antiprion Lead Compounds. (2013) (11)
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- Nerve growth factor induces gene expression of the prion protein and beta-amyloid protein precursor in the developing hamster central nervous system. (1990) (10)
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- Energetische Störung des Kationentransports als Ursache des intrazellulären Hirnödems (1970) (8)
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- 15 Some Strategies and Methods for the Study of Prions (1999) (4)
- Water-Soluble Iridium Photoredox Catalyst for the Trifluoromethylation of Biomolecule Substrates in Phosphate Buffered Saline Solvent. (2021) (4)
- Fibril-like structures in preparations of scrapie prions purified from hamster brain. (1983) (4)
- Tau aggregates are RNA-protein assemblies that mis-localize multiple nuclear speckle components (2021) (4)
- Deciphering Prion Diseases with Transgenic Mice (1996) (4)
- Transgenic mouse brains for the evaluation and quality control of BSE tests (2007) (4)
- Relationship of acidemia to cerebral edema. (1965) (4)
- Prion biology (2011) (4)
- The burden of proof in linking AIDS to scrapie (1987) (4)
- BRAIN PRPSC IN HAMSTER IS SPATIALLY AND TEMPORALLY RELATED TO SCRAPIE HISTOPATHOLOGY AND INFECTIVITY TITER (1988) (3)
- Genetic control of prion replication. (1987) (3)
- Scrapie prions: a three-dimensional model of an infectious fragment. (1996) (3)
- Trans-channel fluorescence learning improves high-content screening for Alzheimer’s disease therapeutics (2021) (3)
- b -Amyloid Prions and the Pathobiology of Alzheimer’s Disease (2017) (3)
- A novel vector for transgenesis in the rat CNS (2017) (3)
- Prion protein — mediator of toxicity in multiple proteinopathies (2020) (3)
- Molecular Biological Studies of Prion Disorders in Humans and Animals (1992) (3)
- Matrix-Assisted Laser Desorption/Ionization Mass Spectrometry of Membrane Proteins: The Scrapie Prion Protein (1993) (3)
- Prions Causing Scrapie and Creutzfeldt-Jakob Disease (1986) (3)
- Biochemistry and gentics of prion proteins (1995) (2)
- Molecular and Genetic Basis of Prion Diseases (1998) (2)
- The prion folding problem . Review article (1998) (2)
- Molecular genetics and biophysics of prions. (1995) (2)
- , M A content and lacking scrapie infectivity . spherical particles having high alpha-helical Disruption of prion rods generates 10-nm (1996) (2)
- Guinea Pig Prion Protein Supports Rapid Propagation of Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease Prions (2016) (2)
- Solid-state NMR studies of the prion protein HI fragment (2002) (2)
- Disinfection of prions (2007) (2)
- Slow Viruses and Prions (1985) (2)
- Biogenesis and transmembrane orientation of the cellular isoform of the scrapie prion protein [published errratum appears in Mol Cell Biol 1987 May;7(5):2035] (1987) (2)
- Aβ and tau prions feature in the neuropathogenesis of Down syndrome (2022) (2)
- A national strategy for development of effective methods for the prevention and treatment of Alzheimer's disease and related neurodegenerative disorders (1994) (2)
- Stacked binding of a small molecule PET tracer to Alzheimer’s tau paired helical filaments (2022) (2)
- An introduction to prion research (1992) (2)
- Human Slow Infections Caused by Prions (1989) (2)
- MODULATION OF γ‐GLUTAMYL TRANSPEPTIDASE ACTIVITY FROM BOVINE CHOROID PLEXUS (1978) (2)
- Correction for Watts et al., Bioluminescence imaging of Aβ deposition in bigenic mouse models of Alzheimer's disease (2015) (2)
- 18 Therapeutic Approaches to Prion Diseases (2004) (2)
- Multiple system atrophy prions transmit neurological disease to mice expressing wild-type human α-synuclein (2022) (2)
- The conformational consequences of mutations to the H1 helix of the prion protein explored by molecular dynamics simulations (1996) (1)
- Guam ALS-PDC is a distinct double-prion disorder featuring both tau and Aβ prions (2023) (1)
- Widening Spectrum of Prions Causing Neurodegenerative Diseases (2013) (1)
- Failure to transmit disease from gray tremor mutant mice (1997) (1)
- Reflections on kuru (2008) (1)
- Synthetic A beta prion strains producing distinct amyloid deposits in the brains of transgenic mice (2014) (1)
- Aβ and Tau Prions Causing Alzheimer's Disease. (2023) (1)
- Adenosine 3 ' : 5 '-Cyclic Monophosphate Control of the Enzymes of Glutamine Metabolism in Escherichia coli ( glutaminases / glutamate synthase / glutamine synthetase / glutamate dehydrogenase / cyclie AMP ) (1)
- THE MOUSE PRION GENE COMPLEX AND SUSCEPTIBILITY TO TRANSMISSIBLE NEURODEGENERATIVE DISEASES (1988) (1)
- THE SITES OF PrPSc DEPOSITION IN THE BRAIN ARE PRION STRAIN SPECIFIC (1993) (1)
- Partial purification and kinetics of gamma-glutamyl transpeptidase from bovine choroid plexus. (1978) (1)
- hamster brain Purification and properties of the cellular prion protein from Syrian (2002) (1)
- Analysis of proteome dynamics in themouse brain (vol 107, pg 14508, 2010) (2014) (1)
- Prion Amyloids in Scrapie and Creutzfeldt-Jakob Disease (1986) (1)
- SCRAPIEPRIONS CAUSE DECREASED MEMBRANE FLUIDITY IN CULTURED CELLS: 20 (1995) (1)
- SPORADIC OCCURRENCE OF FATAL THALAMIC INSOMNIA WITHOUT A PRION PROTEIN GENE MUTATION (1996) (1)
- MOLECULAR GENETICS OF THE MOUSE PRION GENE COMPLEX (1988) (1)
- ELECTROSPRAY MASS SPECTROMETRY OF THE GLYCOSYLINOSITOL PHOSPHOLIPID OF THE SCRAPIE PRION PROTEIN (1992) (1)
- diseases share a common pathway of neurodegeneration (1999) (1)
- Propagation of tau prions from Alzheimer's disease and chronic traumatic encephalopathy patients in cultured cells (2017) (1)
- Kinetics of D-glucose transport into renal membrane vesicles: measurements using a vacuum manifold apparatus. (1981) (1)
- Carotidynia (1977) (1)
- Conditional control of human wildtype and mutated [A30P] alpha-synuclein in a mouse model of Parkinson's disease (2005) (1)
- Tolerance of the vestibular apparatus of the hypothermic hamster to 840 g acceleration. (1965) (1)
- Creutzfeldt-Jakob disease : recent developments (2017) (1)
- MAD COW (2004) (1)
- Cellular immune interactions of splenocytes from scrapie- -infected mice. Abstr. (1977) (1)
- P3-378 Evaluation of the efficacy of quinacrine in animal models of prion disease (2004) (1)
- Linkage of the indiana kindred variant of Gerstmann-Sträussler-Scheinker disease to a mutation in the prion protein gene ( PRNP ) (1992) (1)
- Prions Causing Transmissible Neurodegenerative Diseases (1990) (1)
- Structure and Biology of Scrapie Prions (1985) (1)
- A β prions and the pathobiology of Alzheimer’s disease (2017) (1)
- TRANSGENIC MICE EXPRESSING HAMSTER PRION PROTEIN PRODUCE SPECIES -SPECIFIC SCRAPIE INFECTIVITY AND AMYLOID PLAQUES: 152 (1990) (1)
- PL01-MO-01 Biology of Prion diseases – lessons for other neurodegenerative disorders (2009) (1)
- Prion Biology and Diseases — Fatal Conformations of Proteins during a Journey from Heresy to Orthodoxy (1998) (1)
- Biosafety of prion diseases. (2006) (1)
- Vacuum manifold for rapid assay of enzymes using radioactive tracers and ion exchange chromatography. (1971) (1)
- 2010 Award Recipients - Stephen J. DeArmond, MD, PhD and Samuel K. Ludwin, MB, BCh (2010) (0)
- Konditionelle Expression des humanen alpha-Synukleins in einem Mausmodell für den Morbus Parkinson (2005) (0)
- Characterisation of a conditional mouse-model of Parkinson's disease (2006) (0)
- Prions Causing Neurodegenerative Diseases (1990) (0)
- Introduction and Oral presentations (2011) (0)
- Chemical synthesis, purification and characterization of a 107 residue fragment of the prion protein (2002) (0)
- Transmission and Detection of Prions in Feces. Commentary (2008) (0)
- Improving therapeutic efficacy of 2-aminothiazoles in PrP prion disease (2015) (0)
- Detecting prions in a sample and prion preparation and transgenic animal used for same (1997) (0)
- Sedimentation properties of the scrapie agent ( subcellular fractionation / differential centrifugation / viral agent titration ) (0)
- Scrapie Prions, Amyloid Plaques, and a Possible Link with Alzheimer’s Disease (1987) (0)
- Neurologische Erkrankungen Teil 17 (2016) (0)
- PRIONS | Human and Animal (1999) (0)
- Separation and properties of cellular and scrapie prion proteins (brain subcEllular fractionation/membrane protein immunoblotting/detergent solubilization/amyloid rod formation/slow infections) (2016) (0)
- 11. Jousting with the Press (2017) (0)
- 8 Transgenetic Investigations of the Species Barrier and Prion Strains (1999) (0)
- Silver Benzoate Facilitates the Copper-Catalyzed C–N Coupling of Iodoazoles with Aromatic Nitrogen Heterocycles (2021) (0)
- Removing prions from blood, plasma and other fluids. (1999) (0)
- NMR STRUCTURE OF MOUSE DOPPEL 51-157 (2001) (0)
- New tests can rapidly identify the presence of dangerous prions—the agents responsible for the malady—and several compounds offer hope for treatment (2004) (0)
- 3 Biologie neurologischer Erkrankungen 444 e (2016) (0)
- Erratum to (2011) (0)
- Editorial Board (2011) (0)
- Abstracts of papers presented at the 1992 Meeting on Molecular Biology of Aging, April 22-April 26, 1992 (1992) (0)
- Fusion of prion protein to green fluorescent protein facilitates subcellular localization (1997) (0)
- Structural Characterization of Amyloids Comprised of Anchorless Prion Proteins (2010) (0)
- Monoclonal Antibodies to Prion Proteins Conformation-Dependent High-Affinity (2010) (0)
- Reprint Acknowledgments (2020) (0)
- Dosed for specific strains of conformations of a protein related to various diseases. (1999) (0)
- Protein Structure and Biology (2013) (0)
- Pharmacokinetics and Metabolism of 2-Aminothiazoles with Antiprion Activity in Mice (2013) (0)
- RESISTANCE OF THE SCRAPIE AGENT TO PSORALENS (1983) (0)
- The Molecular Basis of Cellular Dysfunction in Prion Diseases (1998) (0)
- Chapter 16 – PRIONS—STRUCTURE, BIOLOGY, AND DISEASES (1985) (0)
- A novel vector for transgenesis in the rat CNS (2017) (0)
- CHEMICAL AND BIOLOGICAL PROPERTIES OF THE PRION PROTEIN AND ITS CELLULAR ISOFORM (1986) (0)
- Correction for “ Recovery of a top predator mediates negative eutrophic effects on seagrass (2014) (0)
- Conformational changes in the prion protein can be simulated with synthetic peptides (1995) (0)
- The Story of Glutamine Synthetase Regulation (2001) (0)
- Molecular Biology and Genetics of Human Prion Diseases and PrP Amyloid Plaque Formation (1994) (0)
- Replication of multiple system atrophy prions in primary astrocyte cultures from transgenic mice expressing human α-synuclein (2019) (0)
- SCREENING TEST prion proteins in the conformation associated with enfermadad. (1999) (0)
- 6 Doppel, a New PrP-like Mammalian Protein (2004) (0)
- S1-03-04 Transmission studies of mouse synthetic prions (2006) (0)
- Subject Index to Volume 15 (2001) (0)
- Evaluation ofaClass IIIBiological Safety Cabinet for Enclosure ofanUltracentrifuge (1979) (0)
- Regional mapping of prion proteins in brain ( / A 4-amyloid protein / brain mapplng / Creutzfeldt-Jakob disease / hloblots ) (0)
- The molecular basis for transmission of human prion strains (2009) (0)
- Abstract 2. Prion diseases (1985) (0)
- Solution Structure of a Prion Protein: Implications for Infectivity (1998) (0)
- Pathogenic Linkage between Prion and Alzheimer's Disease (2013) (0)
- Synthetic beta-amyloid prions (2013) (0)
- Biochemistry and Genetics of Prion Proteins (1995) (0)
- The Plain and the Ugly Prion Infected Neuronal Tissue in an Experimental Animal Model; An Electron Microscopic Study. (2000) (0)
- Relationship of aluminosilicates to CNS degenerative disorders (1986) (0)
- Are neurodegerative processes in SCA3 reversible? A study using transgenic mouse models (2007) (0)
- Biophysical Studies on Structure Structural Transitions and Infectivity of the Prion Protein (1998) (0)
- COMPARATIVE NEUROPATHOLOGY, NEUROCHEMISTRY AND MOLECULAR BIOLOGY OF SCRAPIE IN SYRIAN, ARMENIAN AND CHINESE HAMSTERS: 89 (1990) (0)
- 10. Distinct prion protein mutations are found in different forms of Gerstmann-Sträussler-Scheinker dyndrome (1991) (0)
- Correction for Watts et al., Bioluminescence imaging of Aβ deposition in bigenic mouse models of Alzheimer's disease (2014) (0)
- 87 AUTORADIOGRAPHIC EVIDENCE OF PrP BINDING SITES IN THE HIPPOCAMPUS (1990) (0)
- The neuropathology of transgenic mice expressing prion protein constructs (1994) (0)
- THE GREEN FLUORESCENT PROTEIN (2008) (0)
- What history tells us The progressive construction of a mechanism for prion diseases (2007) (0)
- Chemical optimization of a novel class of orally available small molecules targeting Prion disease (2010) (0)
- Remembering Radoslav Andjus (2005) (0)
- The New England Journal of Medicine SHATTUCK LECTURE - NEURODEGENERATIVR DISEASES AND PRIONS (2004) (0)
- Appendix Prion Protein (PrP) and Doppel (Dpl) Sequences (2004) (0)
- IMMUNO-GOLD LABELING OF FILAMENTOUS STRUCTURES ANTIGENICALLY RELATED TO THE PRION PROTEIN WITH SCRAPIE INFECTED HAMSTER BRAINS (1986) (0)
- Large-Scale Sequencing of Human, Mouse, and Sheep Prion Protein Genes (1998) (0)
- Citations must default to the online publication date (2018) (0)
- Analysis of expression patterns in the brains of tet-off promoter mice (2004) (0)
- Molecular Biology & Genetics of Prions (2000) (0)
- FURTHER ULTRASTRUCTURAL STUDIES OF SCRAPIE ASSOCIATED PARTICLES (1978) (0)
- A Window Into the Brain: Dementia Risk Factors and Cognitive Functioning (2015) (0)
- EMail from Stanley Prusiner to Louis Sokoloff (2015) (0)
- Scrapie Prions and Degenerative Diseases (1984) (0)
- EXPERIMENTAL MOUSE SCRAPIE; ELECTRON MICROSCOPIC OBSERVATIONS: 8 (1977) (0)
- Analysis of an inducible mouse model for spinocerebellar ataxia type 3 (2005) (0)
- Creutzfeldt-Jakob disease prion propagation in human iPS cells-derived astrocytes (2016) (0)
- Structural studies of the scra electron crystallography (2016) (0)
- P Faculty (2017) (0)
- Chemically Engineering the Prion Protein Using Stepwise SPPS and Expressed Protein Ligation (2001) (0)
- Oral session 1: General neurology (2005) (0)
- Letter from Stanley Prusiner to Louis Sokoloff (1977) (0)
- Biosynthesis of the Scrapie Prion Protein in Scrapie-Infected Cells (1993) (0)
- Conditional control of human wild-type and Parkinson's disease-associated mutant alpha-synuclein in transgenic mouse brain (2004) (0)
- Molecular Biology of Prion Propagation (1998) (0)
- AGED ALZHEIMER’S DISEASE BRAINS EXHIBIT NUMEROUS Aβ BUT ONLY FEW TAU PRIONS (2018) (0)
- Prions causing Alzheimer's and Parkinson's‐Quest for Effective Therapeutics (2015) (0)
- Properties of the Prion Proteins in Creutzfeldt Jakob Disease Patients Heterozygous for the E200K Mutation (1998) (0)
- Commentary Inherited prion diseases (0)
- Pathogenesis of Prion Diseases (2007) (0)
- Prion and Prion-like Diseases in Humans (2013) (0)
- Infectious recombinant prions : In vitro generation and propagation of different strains (0)
- Antibodies as Therapeutic Agents for Prion Disease (2003) (0)
- seRA.PIE PRIONS (1989) (0)
- simple model of protein folding Conformational propagation with prion-like characteristics in a (2000) (0)
- Japan agrees on target for climate curbs (1997) (0)
- Leaf powder of Lawsonia inermis (L) in acetone for the topical application to the fifth instar larvae of silkworm, Bombyx mori (L). (2020) (0)
- [Energetic disturbance of cation transport as a cause of intracellular brain edema]. (1970) (0)
- Time-controlled Transcardiac Perfusion Crosslinking (tcTPC): A Novel Tool for the In Vivo Study of Protein Interactions in Complex Tissues (2003) (0)
- The mode of action of neuropeptides and its relevance for brain diseases (2000) (0)
- CRYSTAL STRUCTURE OF THE ANTI-PRION FAB 3F4 IN COMPLEX WITH ITS PEPTIDE EPITOPE (2000) (0)
- Preface/Front Matter (1999) (0)
- Scrapie and Creutzfeldt-Jakob dise physical properties and antigenic d (slow infections/prion diseases/immunoblots/subviral pathogens/prot (2016) (0)
- CRYSTAL STRUCTURE OF THE ANTI-PRION FAB 3F4 (2000) (0)
- Modeling Multiple System Atrophy Prion Propagation in Astrocytes From Transgenic Mice (2018) (0)
- Table of Contents (1996) (0)
- PRION PROTEIN STANDARD AND METHOD OF MAKING SAME 75 Inventor : (0)
- Inducible expression of ataxin-3 in a transgenic mouse model for Spinocerebellar Ataxia Type 3 (2006) (0)
- Prions, once dismissed as an impossibility, have now gained wide recognition as extraordinary agents that cause a number of infectious, genetic and spontaneous disorders (1995) (0)
- IN SITU IDENTIFICATION OF SCRAPIE PRION PROTEINS IN THE CYTOPLASM OF PERSISTENTLY-INFECTED CULTURED CELLS: 151 (1990) (0)
- 16 Some Strategies and Methods for the Study of Prions (2004) (0)
- Carotidynia or carotid artery aneurysm? (1979) (0)
- Brain-derived and synthetic Aβ aggregates are prions (2012) (0)
- Amyloids in neurodegenerative diseases:immunocytochemical studies (1990) (0)
- Prion diseases (1997) (0)
- Abnormalities of the Plasma Membrane and Endocytosis in Prion-Infected Cell Lines: (1996) (0)
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