Molecular biology and pathogenesis of prion diseases

Novel Proteinaceous Infectious Particles Cause Scrapie

1982 • 4,972 citations

A new variant of Creutzfeldt-Jakob disease in the UK

1996 • 2,690 citations

Seeding “one-dimensional crystallization” of amyloid: A pathogenic mechanism in Alzheimer's disease and scrapie?

1993 • 2,146 citations

Mice devoid of PrP are resistant to scrapie

1993 • 2,058 citations

Molecular Biology of Prion Diseases

1991 • 1,978 citations

Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein

1992 • 1,632 citations

A cellular gene encodes scrapie PrP 27-30 protein

1985 • 1,494 citations

Identification of a Protein That Purifies with the Scrapie Prion

1982 • 1,260 citations

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

1996 • 1,251 citations

Scrapie prion protein contains a phosphatidylinositol glycolipid

1987 • 1,104 citations

A novel progressive spongiform encephalopathy in cattle

1987 • 1,062 citations

Role of the Chaperone Protein Hsp104 in Propagation of the Yeast Prion-Like Factor [ psi ⁺ ]

1995 • 1,036 citations

A protease-resistant protein is a structural component of the Scrapie prion

1983 • 919 citations

Unconventional Viruses and the Origin and Disappearance of Kuru

1977 • 897 citations

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

1990 • 874 citations

Cell-free formation of protease-resistant prion protein

1994 • 872 citations

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

1995 • 835 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

Does the Agent of Scrapie Replicate without Nucleic Acid ?

1967 • 671 citations

Altered circadian activity rhythms and sleep in mice devoid of prion protein

1996 • 655 citations

The scrapie-associated form of PrP is made from a cell surface precursor that is both protease- and phospholipase-sensitive.

1991 • 627 citations

Transmission dynamics and epidemiology of BSE in British cattle

1996 • 601 citations

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 • 541 citations

CREUTZFELDT-JAKOB DISEASE VIRUS ISOLATIONS FROM THE GERSTMANN-STRÄUSSLER SYNDROME

1981 • 534 citations

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

1990 • 510 citations

Slow transmissible diseases of the nervous system

1979 • 503 citations

Purification and structural studies of a major scrapie prion protein

1984 • 497 citations

Identification of scrapie prion protein-specific mRNA in scrapie-infected and uninfected brain

1985 • 490 citations

Structural Clues to Prion Replication

1994 • 469 citations

Linkage of prion protein and scrapie incubation time genes

1986 • 405 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

Conformational transitions, dissociation, and unfolding of scrapie amyloid (prion) protein.

1993 • 369 citations

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

1993 • 331 citations

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

1992 • 276 citations

Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice.

1996 • 253 citations

Prion diseases of humans and animals

1996 • 238 citations

Fibrils from brains of cows with new cattle disease contain scrapie-associated protein

1988 • 233 citations

High-Level Expression and Characterization of a Purified 142-Residue Polypeptide of the Prion Protein

1996 • 188 citations

Conformational Transformations in Peptides Containing Two Putative α-Helices of the Prion Protein

1995 • 178 citations

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

1992 • 168 citations

Creutzfeldt-Jakob Disease: Focus among Libyan Jews in Israel

1974 • 152 citations

Scrapie prion liposomes and rods exhibit target sizes of 55,000 Da

1988 • 136 citations

scrapie Amyloid (Prion) Protein Has the Conformational Characteristics of an Aggregated Molten Globule Folding Intermediate

1994 • 113 citations

Analysis of the prion protein gene in thalamic dementia

1992 • 99 citations

Immunologic and Molecular Biologic Studies of Prion Proteins in Bovine Spongiform Encephalopathy

1993 • 90 citations

Experimental transmission of BSE and scrapie to the common marmoset

1993 • 88 citations

Geographical distribution of cases of Creutzfeldt-Jakob disease in England and Wales 1970-84.

1990 • 65 citations

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

1991 • 47 citations

Experimental scrapie in Cheviot and Suffolk sheep

1969 • 23 citations