New variant prion protein in a Japanese family with Gerstmann-Sträussler syndrome

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

1989 • 834 citations

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

1992 • 680 citations

Fatal Familial Insomnia, a Prion Disease with a Mutation at Codon 178 of the Prion Protein Gene

1992 • 588 citations

Human prion diseases

1994 • 325 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

Mutant prion proteins in Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles

1992 • 240 citations

Über eine eigenartige hereditär- familiäre Erkrankung des Zentralnervensystems

1954 • 207 citations

An Amber Mutation of Prion Protein in Gerstmann-Sträussler Syndrome with Mutant PrP Plaques

1993 • 191 citations

Novel Missense Variants of Prion Protein in Creutzfeldt-Jakob Disease or Gerstmann-Sträussler Syndrome

1993 • 170 citations

A new inherited prion disease (PrP‐P105L mutation) showing spastic paraparesis

1993 • 105 citations

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

1990 • 93 citations

Human prion diseases with variant prion protein

1994 • 91 citations

Creutzfeldt-Jakob disease with codon 129 polymorphism (Valine): a comparative study of patients with codon 102 point mutation or without mutations

1992 • 58 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