Prion Strain-Dependent Differences in Conversion of Mutant Prion Proteins in Cell Culture

Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins.

1993 • 2,237 citations

Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD

1996 • 1,740 citations

NMR structure of the mouse prion protein domain PrP(121–231)

1996 • 1,251 citations

Eight prion strains have PrPSc molecules with different conformations

1998 • 1,236 citations

Prion Protein Biology

1998 • 913 citations

Evidence for the Conformation of the Pathologic Isoform of the Prion Protein Enciphering and Propagating Prion Diversity

1996 • 857 citations

Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein

1995 • 835 citations

Secondary structure analysis of the scrapie-associated protein PrP 27-30 in water by infrared spectroscopy

1991 • 812 citations

Conformational variations in an infectious protein determine prion strain differences

2004 • 782 citations

Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques

1989 • 696 citations

Fatal Familial Insomnia and Familial Creutzfeldt-Jakob Disease: Disease Phenotype Determined by a DNA Polymorphism

1992 • 680 citations

Separation and properties of cellular and scrapie prion proteins.

1986 • 619 citations

Genesis and Variability of [PSI] Prion Factors in Saccharomyces cerevisiae

1996 • 536 citations

Non-genetic propagation of strain-specific properties of scrapie prion protein

1995 • 517 citations

Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation

1997 • 515 citations

RNA molecules stimulate prion protein conversion

2003 • 494 citations

Protein-only transmission of three yeast prion strains

2004 • 473 citations

Biochemical and physical properties of the prion protein from two strains of the transmissible mink encephalopathy agent

1992 • 440 citations

Identification of five allelic variants of the sheep PrP gene and their association with natural scrapie

1995 • 424 citations

Sulfated polyanion inhibition of scrapie-associated PrP accumulation in cultured cells

1993 • 391 citations

The disease characteristics of different strains of scrapie in Sinc congenic mouse lines: implications for the nature of the agent and host control of pathogenesis

1991 • 385 citations

A Critical Role for Amino-Terminal Glutamine/Asparagine Repeats in the Formation and Propagation of a Yeast Prion

1998 • 369 citations

The state of the prion

2004 • 343 citations

A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform 1 1Edited by M. Yaniv

1997 • 340 citations

Strain-dependent Differences in β-Sheet Conformations of Abnormal Prion Protein

1998 • 313 citations

Homozygosity for prion protein alleles encoding glutamine-171 renders sheep susceptible to natural scrapie.

1994 • 260 citations

TSE strain variation

2003 • 259 citations

Fibril Conformation as the Basis of Species- and Strain-Dependent Seeding Specificity of Mammalian Prion Amyloids

2005 • 253 citations

Prions as Protein-Based Genetic Elements

2002 • 249 citations

Prion protein NMR structure and familial human spongiform encephalopathies

1998 • 245 citations

Strain‐specified relative conformational stability of the scrapie prion protein

2001 • 245 citations

Scrapie strain variation and mutation

1993 • 240 citations

Temporary and Permanent Modifications to a Single Strain of Mouse Scrapie on Transmission to Rats and Hamsters

1987 • 240 citations

Successful Transmission of Three Mouse-Adapted Scrapie Strains to Murine Neuroblastoma Cell Lines Overexpressing Wild-Type Mouse Prion Protein

2000 • 234 citations

Sulfated glycans and elevated temperature stimulate PrPSc-dependent cell-free formation of protease-resistant prion protein

2001 • 231 citations

The Genomic Identity of Different Strains of Mouse Scrapie Is Expressed in Hamsters and Preserved on Reisolation in Mice

1989 • 222 citations

Recombinant scrapie-like prion protein of 106 amino acids is soluble

1996 • 175 citations

Interactions between heterologous forms of prion protein: Binding, inhibition of conversion, and species barriers

2000 • 168 citations

Introduction to the transmissible spongiform encephalopathies or prion diseases

2003 • 168 citations

Mapping the early steps in the pH-induced conformational conversion of the prion protein

2001 • 167 citations

Codon 219 lys allele of PRNP is not found in sporadic Creutzfeldt‐Jakob disease

1998 • 160 citations

Scrapie strains maintain biological phenotypes on propagation in a cell line in culture

2001 • 151 citations

The 37 kDa/67 kDa laminin receptor is required for PrP^Sc propagation in scrapie‐infected neuronal cells

2003 • 149 citations

Cellular Heparan Sulfate Participates in the Metabolism of Prions

2003 • 135 citations

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

2003 • 133 citations

Multiple Amino Acid Residues within the Rabbit Prion Protein Inhibit Formation of Its Abnormal Isoform

2003 • 128 citations

Scrapie agent: prions or virinos?

1982 • 128 citations

Sulfated glycosaminoglycans in amyloid plaques of prion diseases

1989 • 123 citations

Supporting the structural basis of prion strains: induction and identification of [PSI] variants11Edited by F. E. Cohen

2001 • 113 citations

Prions: proteins as genes and infectious entities

2004 • 86 citations

The importance of the disulfide bond in prion protein conversion

1998 • 83 citations

Discriminating Scrapie and Bovine Spongiform Encephalopathy Isolates by Infrared Spectroscopy of Pathological Prion Protein

2004 • 79 citations

Reconstitution of Prion Infectivity from Solubilized Protease-resistant PrP and Nonprotein Components of Prion Rods

2001 • 62 citations

The PNM2 mutation in the prion protein domain of SUP35 has distinct effects on different variants of the [ PSI + ] prion in yeast

1999 • 53 citations

Transmissible Encephalopathies: Speculations and Realities

2003 • 50 citations

Deletion of N-terminal Residues 23–88 from Prion Protein (PrP) Abrogates the Potential to Rescue PrP-deficient Mice from PrP-like Protein/Doppel-induced Neurodegeneration

2003 • 48 citations

A Novel Epitope for the Specific Detection of Exogenous Prion Proteins in Transgenic Mice and Transfected Murine Cell Lines

1999 • 45 citations

Biological and Biochemical Characteristics of Prion Strains Conserved in Persistently Infected Cell Cultures

2005 • 39 citations