Prion Protein Amyloidosis

Novel Proteinaceous Infectious Particles Cause Scrapie

1982 • 4,972 citations

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

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

Scrapie prions aggregate to form amyloid-like birefringent rods

1983 • 1,048 citations

Neurotoxicity of a prion protein fragment

1993 • 937 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

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

1991 • 812 citations

Prion protein is necessary for normal synaptic function

1994 • 802 citations

Sulfated Glycans Stimulate Endocytosis of the Cellular Isoform of the Prion Protein, PrPC, in Cultured Cells

1995 • 702 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

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

1981 • 534 citations

Truncated Forms of the Human Prion Protein in Normal Brain and in Prion Diseases

1995 • 497 citations

Evidence for synthesis of scrapie prion proteins in the endocytic pathway.

1992 • 497 citations

Scrapie prion proteins are synthesized in neurons.

1986 • 422 citations

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

1993 • 369 citations

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

1992 • 327 citations

The major polypeptide of scrapie-associated fibrils (SAF) has the same size, charge distribution and N-terminal protein sequence as predicted for the normal brain protein (PrP).

1986 • 322 citations

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

1994 • 311 citations

A prion protein cycles between the cell surface and an endocytic compartment in cultured neuroblastoma cells

1993 • 311 citations

Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein.

1994 • 281 citations

Vascular variant of prion protein cerebral amyloidosis with tau-positive neurofibrillary tangles: the phenotype of the stop codon 145 mutation in PRNP.

1996 • 275 citations

Synthesis and trafficking of prion proteins in cultured cells.

1992 • 268 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

Synthetic peptides homologous to prion protein residues 106-147 form amyloid-like fibrils in vitro.

1993 • 241 citations

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

1992 • 240 citations

Processing of a cellular prion protein: identification of N- and C-terminal cleavage sites

1993 • 211 citations

Über eine eigenartige hereditär- familiäre Erkrankung des Zentralnervensystems

1954 • 207 citations

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

1992 • 204 citations

The Neuropathology and Epidemiology of Bovine Spongiform Encephalopathy

1995 • 199 citations

Amyloid protein of Gerstmann-Sträussler-Scheinker disease (Indiana kindred) is an 11 kd fragment of prion protein with an N-terminal glycine at codon 58.

1991 • 195 citations

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

1993 • 191 citations

Proposed three-dimensional structure for the cellular prion protein.

1994 • 179 citations

Genetic and Infectious Prion Diseases

1993 • 175 citations

Gerstmann‐Sträussler‐Scheinker disease II. Neurofibrillary tangles and plaques with PrP‐amyloid coexist in an affected family

1989 • 173 citations

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

1993 • 170 citations

A Mutant Prion Protein Displays an Aberrant Membrane Association When Expressed in Cultured Cells

1995 • 160 citations

Immunolocalization of heparan sulfate proteoglycans to the prion protein amyloid plaques of Gerstmann-Straussler syndrome, Creutzfeldt-Jakob disease and scrapie.

1990 • 159 citations

Human Prion Protein cDNA: Molecular Cloning, Chromosomal Mapping, and Biological Implications

1986 • 153 citations

A prion protein variant in a family with the telencephalic form of Gerstmann‐Sträussler‐Scheinker syndrome

1991 • 150 citations

Gerstmann‐Sträussler‐Scheinker Disease and the Indiana Kindred

1995 • 147 citations

Amyloid fibrils in Gerstmann-Sträussler-Scheinker disease (Indiana and Swedish Kindreds) express only PrP peptides encoded by the mutant allele

1994 • 138 citations

The Original Gerstmann‐Sträussler‐Scheinker Family of Austria: Divergent Clinicopathological Phenotypes but Constant PrP Genotype

1995 • 137 citations

Molecular pathology of scrapie‐associated fibril protein (PrP) in mouse brain affected by the ME7 strain of scrapie

1988 • 131 citations

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

1993 • 105 citations

A Neurotoxic Prion Protein Fragment Induces Rat Astroglial Proliferation and Hypertrophy

1994 • 103 citations

Familial dementia with PrP‐positive amyloid plaques

1989 • 94 citations

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

1990 • 93 citations

Gerstmann‐Sträussler‐Scheinker disease. I. Extending the clinical spectrum

1989 • 84 citations

Prion protein preamyloid and amyloid deposits in Gerstmann-Sträussler-Scheinker disease, Indiana kindred.

1992 • 81 citations

Morphogenesis of amyloid plaques in 87V murine scrapie†

1994 • 70 citations

Familial Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles

1994 • 69 citations

Prion disease (PrP‐A117V) presenting with ataxia instead of dementia

1995 • 67 citations

Neurofibrillary tangles of the Indiana kindred of Gerstmann-Stra¨ussler-Scheinker disease share antigenic determinants with those of Alzheimer disease

1990 • 67 citations

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

1995 • 61 citations

Familial cerebral amyloidosis and spongiform encephalopathy.

1982 • 58 citations

A68 is a component of paired helical filaments of Gerstmann-Sträussler-Scheinker disease, Indiana kindred

1993 • 53 citations

ßPP Participates in PrP-Amyloid Plaques of Gerstmann-Sträussler-Scheinker Disease, Indiana Kindred

1993 • 52 citations

Light And Electron Microscopic Neuropathology of Slow Virus Disorders

2019 • 51 citations

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

1991 • 51 citations

SPONGIFORM ENCEPHALOPATHY TRANSMITTED EXPERIMENTALLY FROM CREUTZFELDT-JAKOB AND FAMILIAL GERSTMANN-STRÄUSSLER-SCHEINKER DISEASES

1990 • 47 citations

Gerstmann-Straussler-Scheinker disease in an Alsatian family: clinical and genetic studies.

1992 • 47 citations

A missense mutation at codon 105 with codon 129 polymorphism of the prison protein gene in a new variant of Gerstmann‐Strässler‐Scheinker disease

1993 • 45 citations

CEREBROVASCULAR AMYLOIDOSIS IN SCRAPIE‐AFFECTED SHEEP

1986 • 43 citations

The presence of complements in amyloid plaques of Creutzfeldt-Jakob disease and Gerstmann-Straussler-Scheinker disease.

1984 • 41 citations

Gerstmann-Sträussler-Scheinker Disease (PRNP P102L): Amyloid Deposits Are Best Recognized by Antibodies Directed to Epitopes in PrP Region 90-165

1995 • 40 citations

Gerstmann-Straussler-Scheinker disease with mutation at codon 102 and methionine at codon 129 of PRNP in previously unreported patients

1995 • 39 citations

Gerstmann-Str�ussler syndrome ? A variant type: amyloid plaques and Alzheimer's neurofibrillary tangles in cerebral cortex

1992 • 38 citations

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

1991 • 37 citations

Prion diseases of humans and animals

2010 • 36 citations

Immortality of cell cultures derived from brains of mice and hamsters infected with Creutzfeldt-Jakob disease agent.

1987 • 29 citations

Apolipoprotein E and J immunoreactivity in Gerstmann-Sträussler-Scheinker disease

1994 • 13 citations

ONLY MUTANT PRP PARTICIPATES IN AMYLOID FORMATION IN GERSTMANN-STRÄUSSLER-SCHEINKER DISEASE WITH ALA>VAL SUBSTITUTION AT CODON 117

1995 • 10 citations

No correlation between clinical heterogeneity and codon 129 polymorphism of the prion protein gene (PRNP) in Gerstmann-Straussler-Scheinker Syndrome (GSS) with PRNP codon 102 mutation

1994 • 3 citations

EXPERIMENTAL (HAMSTER) SCRAPIE

1993 • 2 citations

165 Prior protein (PrP) residue 129 Val in Gerstmann-Sträussler-Scheinker (GSS) disease with mutation PRNP P102L

1996 • 1 citations