The primary structure of the prion protein influences the distribution of abnormal prion protein in the central nervous system.

Novel Proteinaceous Infectious Particles Cause Scrapie

1982 • 4,972 citations

Molecular Biology of Prion Diseases

1991 • 1,978 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

Spontaneous Neurodegeneration in Transgenic Mice with Mutant Prion Protein

1990 • 531 citations

Formic acid pretreatment enhances immunostaining of cerebral and systemic amyloids.

1987 • 469 citations

Abnormal isoform of prion protein accumulates in follicular dendritic cells in mice with Creutzfeldt-Jakob disease

1991 • 290 citations

Abnormal isoform of prion proteins accumulates in the synaptic structures of the central nervous system in patients with Creutzfeldt-Jakob disease.

1992 • 271 citations

Pro→Leu change at position 102 of prinon protein is the most common but not the sole mutation related to Gerstmann-Sträussler syndrome

1989 • 268 citations

Prion dementia without characteristic pathology

1990 • 230 citations

Amyloid plaques in Creutzfeldt‐Jakob disease stain with prion protein antibodies

1986 • 175 citations

DIAGNOSIS OF GERSTMANN-STRÄUSSLER SYNDROME IN FAMILIAL DEMENTIA WITH PRION PROTEIN GENE ANALYSIS

1989 • 148 citations

An in-frame insertion in the prion protein gene in familial Creutzfeldt-Jakob disease

1990 • 148 citations

Different scrapie-associated fibril proteins (PrP) are encoded by lines of sheep selected for different alleles of the Sip gene

1991 • 142 citations

Non-hydrophobic extracytoplasmic determinant of stop transfer in the prion protein

1990 • 132 citations

Immunochemical, molecular genetic, and transmission studies on a case of Gerstmann‐Straussler‐Scheinker syndrome

1990 • 93 citations

Three hamster species with different scrapie incubation times and neuropathological features encode distinct prion proteins.

1990 • 86 citations

Immunohistochemical confirmation of Creutzfeldt-Jakob disease with a long clinical course with amyloid plaque core antibodies.

1988 • 81 citations

The sequential development of abnormal prion protein accumulation in mice with Creutzfeldt-Jakob disease.

1992 • 80 citations

Gerstmann‐Sträussler‐Scheinker disease: Immunohistological and experimental studies

1988 • 55 citations

N-terminal sequence of prion protein is also intergrated into kuru plaques in patients with Gerstmann-Stra¨ussler syndrome

1991 • 51 citations

Creutzfeldt‐Jakob disease patients with congophilic kuru plaques have the missense variant prion protein common to Gerstmann‐Sträussler syndrome

1990 • 44 citations

A prion protein missense variant is integrated in kuru plaque cores in patients with Gerstmann‐Sträussler syndrome

1991 • 37 citations

Three Hamster Species with Different Scrapie Incubation Times and Neuropathological Features Encode Distinct Prion Proteins

1990 • 26 citations