Structural Clues to Prion Replication

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

1993 • 2,237 citations

Mice devoid of PrP are resistant to scrapie

1993 • 2,058 citations

Molecular Biology of Prion Diseases

1991 • 1,978 citations

Protein Folding

1992 • 1,601 citations

Scrapie prion protein contains a phosphatidylinositol glycolipid

1987 • 1,104 citations

Scrapie prions aggregate to form amyloid-like birefringent rods

1983 • 1,048 citations

Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication

1990 • 874 citations

Homozygous prion protein genotype predisposes to sporadic Creutzfeldt–Jakob disease

1991 • 871 citations

Linkage of a prion protein missense variant to Gerstmann–Sträussler syndrome

1989 • 834 citations

Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene

1986 • 829 citations

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

1991 • 812 citations

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

1992 • 680 citations

Caveolae: where incoming and outgoing messengers meet.

1993 • 616 citations

Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing

1993 • 578 citations

Spontaneous Neurodegeneration in Transgenic Mice with Mutant Prion Protein

1990 • 531 citations

Scrapie and cellular prion proteins differ in their kinetics of synthesis and topology in cultured cells.

1990 • 510 citations

Distinct prion proteins in short and long scrapie incubation period mice

1987 • 482 citations

Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies.

1993 • 461 citations

Identification of a gene which controls the incubation period of some strains of scrapie agent in mice

1968 • 425 citations

Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins

1994 • 340 citations

Propagation of prions with artificial properties in transgenic mice expressing chimeric PrP genes

1993 • 331 citations

Prion protein biosynthesis in scrapie-infected and uninfected neuroblastoma cells

1989 • 330 citations

Predicted alpha-helical regions of the prion protein when synthesized as peptides form amyloid.

1992 • 327 citations

Fatal familial insomnia and familial Creutzfeldt-Jakob disease: different prion proteins determined by a DNA polymorphism.

1994 • 311 citations

Perturbation of the secondary structure of the scrapie prion protein under conditions that alter infectivity.

1993 • 307 citations

Identification of two biologically distinct strains of transmissible mink encephalopathy in hamsters

1992 • 276 citations

Replication of distinct scrapie prion isolates is region specific in brains of transgenic mice and hamsters.

1992 • 213 citations

Linkage of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease to the prion protein gene

1992 • 204 citations

Thermal stability and conformational transitions of scrapie amyloid (prion) protein correlate with infectivity

1993 • 204 citations

Proposed three-dimensional structure for the cellular prion protein.

1994 • 179 citations

Conversion of truncated and elongated prion proteins into the scrapie isoform in cultured cells.

1993 • 173 citations

INHERITED PRION DISEASE WITH 144 BASE PAIR GENE INSERTION: 1. GENEALOGICAL AND MOLECULAR STUDIES

1992 • 168 citations

Prion isolate specified allotypic interactions between the cellular and scrapie prion proteins in congenic and transgenic mice.

1994 • 154 citations

Attempts to restore scrapie prion infectivity after exposure to protein denaturants.

1993 • 130 citations

Endocytosis of glycophospholipid-anchored and transmembrane forms of CD4 by different endocytic pathways.

1992 • 129 citations

Structure and polymorphism of the mouse prion protein gene.

1994 • 104 citations

Three scrapie prion isolates exhibit different accumulation patterns of the prion protein scrapie isoform.

1993 • 101 citations

Analysis of the prion protein gene in thalamic dementia

1992 • 99 citations

Mutation and polymorphism of the prion protein gene in Libyan Jews with Creutzfeldt-Jakob disease (CJD).

1993 • 97 citations

Primary structure of prion protein may modify scrapie isolate properties.

1989 • 75 citations

Conservation of infectivity in purified fibrillary extracts of scrapie-infected hamster brain after sequential enzymatic digestion or polyacrylamide gel electrophoresis.

1990 • 63 citations

Adenovirus E4-dependent activation of the early E2 promoter is insufficient to promote the early-to-late-phase transition

1991 • 47 citations

Three scrapie prion isolates exhibit different accumulation patterns of the prion protein scrapie isoform.

1993 • 38 citations