Search Results - "prion"

Prions and prion diseases new developments /

Subjects: “…Prion diseases.…”
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Chaperones and prions

Table of Contents: “…Contents: Prion diseases -- Prions as self-propagating amyloids -- Yeast prions as protein-based heritable elements -- Prion detection -- Crucial role of the chaperone protein Hsp104 in the propagation of yeast prions -- Chaperone-mediated fragmentation turns amyloids into infectious or heritable prions -- Effects of the Hsp70 and Hsp40 proteins on yeast prions -- Other modulators of prion formation and propagation: cytoskeleton and ubiquitin system.…”
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Prions and amyloids introduction /

Table of Contents: “…Contents: Definitions of prions and amyloid -- Genetic criteria for a prion -- Prions of mammals, yeast and filamentous fungi -- Prion domains -- Non-amyloid prions.…”
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Prion biology and diseases

Subjects: “…Prions.…”
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Fatal flaws how a misfolded protein baffled scientists and changed the way we look at the brain / by Ingram, Jay

Subjects: “…Prions.…”
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Yeast and fungal prions a help or a hindrance? /

Table of Contents: “…Contents: [URE3] and [PSI+] not found in wild strains indicating they are harmful -- [Het-s] may benefit its host or may result from "meiotic drive" -- Some "prion domains" cannot be prions -- "Prion domains" have non-prion functions -- Rapid evolution of prion domains may protect against infection.…”
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[PIN+] prions beget prions /

Table of Contents: “…Contents: Prions are infectious proteins -- [PIN+] is a prion of Rnq1 - [PIN+] exists as different variants -- Prions can affect the stability of other variants of the same prion, or of heterologous prions.…”
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Mechanism of prion generation in vitro

Table of Contents: “…Contents: Prion propagation in vitro requires PrP plus an incorporated polyanion -- PrPC post-translational modifications influence prion formation -- Zinc and copper inhibit PrPSc amplification.…”
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Mechanisms of yeast prion propagation

Table of Contents: “…Contents: Prions as infectious amyloids -- Yeast [PSI+] prion and Sup35p -- Molecular chaperone Hsp104 -- Guanidine hydrochloride as a prion propagation inhibitor -- Propagons -- Estimating propagon numbers using a stochastic model -- Generation of new propagons -- Propagation-defective mutants (PNM) -- Role of primary sequence information -- Yeast prion propagation: the unanswered questions.…”
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Yeast prions and protein chaperones a help or a hindrance? /

Table of Contents: “…Contents: Role of protein chaperones in protein folding -- Yeast prions as amyloid forms of cellular proteins -- Requirement of chaperone machinery for prion propagation -- Chaperone mutants adversely affect prions and provide insight into chaperone/amyloid interactions.…”
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The genetics and biology of the [Het-s] prion of Podospora

Table of Contents: “…Contents: Mammalian prions -- Fungal prions -- Yeast: [URE3], [PSI+] and [PIN+] -- Filamentous fungi: [Het-s] -- Fungal self non-self recognition -- Heterokaryon incompatibility -- Genetics of [Het-s] -- Maternal inheritance -- Meiotic drive -- In vivo and in vitro aggregation of HET-s protein -- Role of the Hsp104 chaperone in [Het-s] system -- Role of the Hsp104 chaperone in [Het-s] prion propagation -- Podospora anserine.…”
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Molecular structure of amyloid and prion fibrils insights from solid state NMR /

Table of Contents: “…Contents: Overview of solid state NMR methodology -- Structural model for Alzheimer's beta-amyloid fibrils -- Self-propagating polymorphism in amyloid fibrils -- Molecular basis of beta-amyloid polymorphism -- Extension of solid state NMR methods to amylin fibrils and yeast prion fibrils -- Structural basis of the generic nature of amyloid formation.…”
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Propagation and variability of the yeast [PSI+] prion

Table of Contents: “…Contents: Prion -- Yeast prions as non-chromosomal genetic determinants -- Modular structure of the Sup35 protein responsible for the yeast [PSI+] prion -- Role of the Hsp104 chaperone in [PSI+] prion propagation -- Two-level structure of the [PSI+] prion aggregates and methods for studying the size of prion polymers -- Non-prion amyloids in yeast -- Variability of the yeast [PSI+] prion.…”
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Similarities between prion diseases and other neurodegenerative diseases

Table of Contents: “…Contents: Pathology of diseases -- Prion diseases -- The first family with a prion mutation: Creutzfeldt-Jacob disease -- Alzheimer's disease -- 7Mb duplication of locus around APP -- APP probably a locus for "sporadic" Alzheimer's disease -- Diseases with only Lewy bodies: Parkinson's disease and Lewy body dementia -- Iowa kindred structure -- alpha-synuclein -- Diseases with only Tau pathology -- The microtubule associated protein tau -- Evolution of human tau haplotypes -- Tagging SNPs selection -- More complex than just H1/H2 -- Association with PSP is driven by H1c allele -- Primary tauopathies -- "Prion-like " behaviour of A-beta -- Does the "spreading" of diseases reflect a less extreme version of infection? …”
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Predicting TSE transmission

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Generation and propagation of infectious prions by cyclic amplification of protein misfolding

Table of Contents: “…Contents: Prions as infectious proteins and the mechanism of replication -- The rationale and proof of concept for the PMCA technology -- Multiple applications of PMCA -- Generation of infectious prions by PMCA -- Studies of prion strains and species barrier -- Use of PMCA for prion detection and diagnosis.…”
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The structure of amyloid fibrils of the HET-s (218-289) prion as seen by solid-state NMR

Table of Contents: “…Contents: 3D structure is important -- Prion hypothesis -- Prions can form amyloids -- Amyloids -- Cross-beta arrangements give characteristic fiber diffraction patterns -- Solid-state NMR can characterize amyloid fibrils -- Atomic-resolution structure determination by NMR and x-ray -- Distance information in NMR correlation spectra -- The methodology to obtain 3D structures from solid (microcrystalline) samples has recently been developed -- Structure determination is possible -- Nevertheless, amyloids are different -- Isotopic labeling allows to disentangle the intra/inter problem -- HET-s: a functional prion -- Structural information about HET-s (218-289) from earlier measurements -- Chemical shift assignment -- To go further we need distance restraints -- Distance restraints for protein fibrils -- Intramolecular restraints -- Structure calculation -- Structural features of the fibrils -- Comparison with globular proteins.…”
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Transgenic mouse models of prion diseases

Table of Contents: “…Contents: Prions represent a new biological paradigm of protein-mediated information transfer -- Prions are the cause of fatal, transmissible neurodegenerative diseases, often referred to as transmissible spongiform encephalopathies (TSEs) -- Many unresolved issues remain including the exact molecular nature of the prion, the detailed mechanism of prion propagation, and the mechanism by which prion diseases can be both genetic and infectious -- The factors governing the transmission of prions between species remain unclear, in particular the means by which prion strains and PrP primary structure interact to affect inter-species prion transmission -- Dramatic advances in our understanding of prions have occurred because of their transmissibility to experimental animals and the development of transgenic (Tg) mouse models has done much to further our understanding about various aspects of prion biology.…”
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Expanding roles of RNA-binding proteins in neurodegenerative diseases

Table of Contents: “…Contents: Amyotrophic lateral sclerosis (ALS) -- Genetic causes of ALS -- Role of RNA-binding proteins and stress granules in ALS pathogenesis -- Emerging class of RNA-binding proteins with prion-like domains in neurodegenerative disease -- Mechanisms by which C9ORF72 mutations cause ALS.…”
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Neurodegenerative disease expression levels of normal sequence pathogenic proteins contribute to risk of disease /

Table of Contents: “…Contents: Protein pathology of disease -- Loci underlying autosomal dominant neurodegenerative disease -- Prion mutations causing Gerstmann Straussler syndrome and hereditary Creuzfeldt-Jakob disease -- Tau mutations cause autosomal dominant frontal temporal dementia -- Alpha-synuclein mutations cause autosomal dominant Parkinson's disease -- How genetic variability of these genes contributes to the risk of sporadic disease.…”
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