Insoluble wild-type and protease-resistant mutant prion protein in brains of patients with inherited prion disease

scientific article published on January 1996

Insoluble wild-type and protease-resistant mutant prion protein in brains of patients with inherited prion disease is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1038/NM0196-59
P698PubMed publication ID8564843

P50authorStanley B. PrusinerQ155380
P2093author name stringTelling G
Meiner Z
Gabizon R
Halimi M
Kahana I
P2860cites workConversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteinsQ24564014
Acquisition of protease resistance by prion proteins in scrapie-infected cells does not require asparagine-linked glycosylationQ33860629
Prion propagation in mice expressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another proteinQ34294957
Molecular biology of prion diseasesQ34534878
Mutant prion proteins in Gerstmann-Sträussler-Scheinker disease with neurofibrillary tanglesQ34539307
Fatal familial insomnia and familial Creutzfeldt-Jakob disease: different prion proteins determined by a DNA polymorphismQ35138028
Inherited prion diseasesQ35251034
Separation and properties of cellular and scrapie prion proteinsQ35598678
Proposed three-dimensional structure for the cellular prion proteinQ35618317
Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion proteinQ35823555
Perturbation of the secondary structure of the scrapie prion protein under conditions that alter infectivityQ36022813
Synthetic peptides corresponding to different mutated regions of the amyloid gene in familial Creutzfeldt-Jakob disease show enhanced in vitro formation of morphologically different amyloid fibrilsQ36299442
A kinetic model for amyloid formation in the prion diseases: importance of seedingQ36385621
Predicted alpha-helical regions of the prion protein when synthesized as peptides form amyloidQ37302673
The risk of developing Creutzfeldt-Jakob disease in subjects with the PRNP gene codon 200 point mutationQ38939145
Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob diseaseQ39080950
Creutzfeldt-Jakob Disease: Focus among Libyan Jews in IsraelQ39115314
Presence of prion protein in peripheral tissues of Libyan Jews with Creutzfeldt-Jakob diseaseQ39241840
Intracellular accumulation of the cellular prion protein after mutagenesis of its Asn-linked glycosylation sitesQ41723952
An amber mutation of prion protein in Gerstmann-Sträussler syndrome with mutant PrP plaquesQ42601576
Monitoring treatment with aminoglycoside antibioticsQ43709580
Prion protein peptides induce alpha-helix to beta-sheet conformational transitionsQ44458369
Mutation in codon 200 of scrapie amyloid protein gene in two clusters of Creutzfeldt-Jakob disease in SlovakiaQ45007616
Creutzfeldt-Jakob disease virus isolations from the Gerstmann-Sträussler syndrome with an analysis of the various forms of amyloid plaque deposition in the virus-induced spongiform encephalopathiesQ45794286
Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication.Q45979200
Linkage of a prion protein missense variant to Gerstmann-Sträussler syndromeQ46931937
Neurotoxicity of a prion protein fragmentQ48304223
Familial Creutzfeldt-Jakob disease in Chile is associated with the codon 200 mutation of the PRNP amyloid precursor gene on chromosome 20.Q48420015
Sulphate polyanions prolong the incubation period of scrapie-infected hamsters.Q48521519
Fatal familial insomnia and familial Creutzfeldt-Jakob disease: disease phenotype determined by a DNA polymorphismQ48882672
Chemoprophylaxis of scrapie in miceQ53030476
Potent inhibition of scrapie-associated PrP accumulation by congo redQ54131452
Mutation in codon 200 of scrapie amyloid precursor gene linked to Creutzfeldt-Jakob disease in Sephardic Jews of Libyan and non-Libyan originQ59417090
New mutation in scrapie amyloid precursor gene (at codon 178) in Finnish Creutzfeldt-Jakob kindredQ68292026
A prion protein missense variant is integrated in kuru plaque cores in patients with Gerstmann‐Sträussler syndromeQ68292323
The antiviral compound HPA-23 can prevent scrapie when administered at the time of infectionQ71239452
Heparin-like molecules bind differentially to prion-proteins and change their intracellular metabolic fateQ72656295
Dextran Sulphate 500 Delays and Prevents Mouse Scrapie by Impairment of Agent Replication in SpleenQ72739726
Amyloid fibrils in Gerstmann-Sträussler-Scheinker disease (Indiana and Swedish kindreds) express only PrP peptides encoded by the mutant alleleQ93870994
Counselling in general practiceQ93880164
P433issue1
P407language of work or nameEnglishQ1860
P921main subjectprion protein familyQ24724413
P304page(s)59-64
P577publication date1996-01-01
P1433published inNature MedicineQ1633234
P1476titleInsoluble wild-type and protease-resistant mutant prion protein in brains of patients with inherited prion disease
P478volume2

Reverse relations

cites work (P2860)
Q43510340A 7-kDa prion protein (PrP) fragment, an integral component of the PrP region required for infectivity, is the major amyloid protein in Gerstmann-Sträussler-Scheinker disease A117V.
Q57371794A C-terminal-truncated PrP Isoform Is Present in Mature Sperm
Q33952821A protease-resistant prion protein isoform is present in urine of animals and humans affected with prion diseases
Q48550880A wild-type prion protein does not acquire properties of the scrapie isoform when coexpressed with a mutant prion protein in cultured cells
Q30734837Adapting protein solubility by glycosylation. N-glycosylation mutants of Coprinus cinereus peroxidase in salt and organic solutions.
Q59690434Allelic origin of the abnormal prion protein isoform in familial prion diseases
Q42396970CEREBRAL AMYLOID ANGIOPATHY AND ALZHEIMER'S DISEASE.
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Q35915387Chemical chaperones interfere with the formation of scrapie prion protein
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Q35745733Effect of the E200K mutation on prion protein metabolism. Comparative study of a cell model and human brain
Q48168745Epitope scanning indicates structural differences in brain-derived monomeric and aggregated mutant prion proteins related to genetic prion diseases.
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Q33745216Iowa variant of familial Alzheimer's disease: accumulation of posttranslationally modified AbetaD23N in parenchymal and cerebrovascular amyloid deposits
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