|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Neuropathologically, the common denominator is a cerebral prion protein amyloidosis; however, there is significant variability in the pattern of amyloid deposition in regions of the central nervous system among reported families.
|
7767492 |
1995 |
|
Amyloidosis
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The amyloid or prion protein (PrP) genotype showed features typically seen in FFI, with a 178Asn mutation and a 129Met polymorphism.
|
7783865 |
1995 |
|
Amyloidosis
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Gerstmann-Sträussler-Scheinker (GSS) disease is a cerebral amyloidosis linked to mutations of the PRNP gene.
|
7954833 |
1994 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
The term "prion dementia" has been proposed to replace "spongiform encephalopathy", to accommodate the existence of atypical forms of these "prion protein" (PrP) cerebral amyloidoses that may not show spongiform changes in the brain.
|
8093741 |
1993 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Alzheimer disease and the prion disorders amyloid beta-protein and prion protein amyloidoses.
|
8101988 |
1993 |
|
Amyloidosis
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
All amyloid precipitates apparent at the light microscopic level immunostained for PrP.
|
8310806 |
1993 |
|
Amyloidosis
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
In conclusion, factors in addition to the PRNP genotype at codons 102 and 129 must play a role in determining clinicopathological characteristics of this inherited brain amyloidosis.
|
8520719 |
1995 |
|
Amyloidosis
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Vascular variant of prion protein cerebral amyloidosis with tau-positive neurofibrillary tangles: the phenotype of the stop codon 145 mutation in PRNP.
|
8570627 |
1996 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Prion protein amyloidosis.
|
8737929 |
1996 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Immunohistochemical studies showed that in addition to amyloid, these patients accumulate PrP deposits which are neither fluorescent nor birefringent when stained with thioflavin S and Congo red.
|
8939199 |
1996 |
|
Amyloidosis
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Gerstmann-Sträussler-Scheinker disease (GSS) is a cerebral amyloidosis associated with mutations in the prion protein (PrP) gene (PRNP).
|
11087738 |
2001 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Hereditary prion protein amyloidoses.
|
12733425 |
2003 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
FDDNP binds to PrP-amyloid in postmortem human specimens, but has not been reported in vivo in prion disease.
|
17636066 |
2007 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Association between deposition of beta-amyloid and pathological prion protein in sporadic Creutzfeldt-Jakob disease.
|
18349519 |
2008 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Amyloid-beta oligomers bind with nanomolar affinity to PrP(C), but the interaction does not require the infectious PrP(Sc) conformation.
|
19242475 |
2009 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Aggregation and amyloid fibril formation induced by chemical dimerization of recombinant prion protein in physiological-like conditions.
|
19710507 |
2009 |
|
Amyloidosis
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
In this report, we describe the clinical, histopathological and pathological prion protein (PrP(Sc)) characteristics of two Dutch patients carrying novel adjacent stop codon mutations in the C-terminal part of PRNP, resulting in either case in hereditary prion protein amyloidoses, but with strikingly different clinicopathological phenotypes.
|
19911184 |
2010 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Because prion protein PrP-(23-98) was recently found to polymerize into amyloid-like and proteinase K-resistant spherical aggregates in the presence of NADPH plus copper ions, we tested to determine whether calreticulin (CRT) inhibits PrP-(23-98) aggregation in vitro.
|
21821925 |
2011 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Structural and mechanistic commonalities of amyloid-β and the prion protein.
|
21862871 |
2013 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
For synthetic amyloid-β42, small oligomeric species showed prominent binding to PrP(C), whereas in Alzheimer's disease brains larger protein assemblies containing amyloid-β42 bound efficiently to PrP(C).
|
24519981 |
2014 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Soluble Amyloid-β oligomers (Aβo) can trigger Alzheimer disease (AD) pathophysiology by binding to cell surface cellular prion protein (PrP(C)).
|
25148681 |
2014 |
|
Amyloidosis
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
This heterologous association induced aggregation of monomeric PrP and modified the structural properties of PrP amyloid fibrils.
|
25853328 |
2015 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Prion-Protein-interacting Amyloid-β Oligomers of High Molecular Weight Are Tightly Correlated with Memory Impairment in Multiple Alzheimer Mouse Models.
|
26018073 |
2015 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
The native state of prion protein (PrP) directly inhibits formation of PrP-amyloid fibrils in vitro.
|
28373719 |
2017 |
|
Amyloidosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Targeting glutamatergic and cellular prion protein mechanisms of amyloid β-mediated persistent synaptic plasticity disruption: Longitudinal studies.
|
28390893 |
2017 |