Search Results - "prion"

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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Endoplasmic reticulum stress in neurodegenerative diseases

Table of Contents: “…Contents: Neurodegenerative disease -- Misfolded aggregates deposited in the brain -- Research strategy -- Prion diseases -- Two conformations of the prion protein -- Experimental scrapie model in mice -- Upregulation of ER chaperones -- ER-stress and UPR in human CJD -- PrP interaction with ER chaperones in brain -- PrPSc induction of ER-stress and neuronal death -- Calcineurin properties -- A novel treatment for prion diseases.…”
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Novel approaches to treating symptoms and slowing the progress of neurodegenerative diseases

Table of Contents: “…Contents: Brief overview of dementia -- Current treatments for Alzheimer's disease -- Role of muscarinic receptors in learning and memory -- Using prion disease as a model -- Effects of muscarinic receptor drugs on learning and memory deficits in prion disease -- Types of drugs for different stages of Alzheimer's disease.…”
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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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Food safety contaminants and toxins /

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The dark side of amyloid PMEL, a natural amyloid in melanosome biogenesis /

“…Biomedical & life sciences collection. Prions and amyloids : self-propagating protein structures in mammals, yeast and fungi.…”
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Cytoplasmic epigenetics proteins acting as genes /

Table of Contents: “…Contents: Prions (infectious proteins) include several self-propagating amyloids of S. cerevisiae and Podospora anserina and a self-activating enzyme of S. cerevisiae -- these non-chromosomal genetic elements are genes composed of protein, just as nucleic acids can catalyse enzymatic reactions -- the amyloid-based prions [PSI+] and [URE3] are diseases of yeast, but the [Het-s] prion of Podospora carries out a normal function for that organism, heterokaryon incompatibility.…”
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Hsp104 a specialized chaperone for protein disaggregation /

Table of Contents: “…Contents: Heat shock -- Molecular chaperones and thermotolerance -- Protein aggregation and refolding -- Prions in mammals and yeast -- Molecular chaperones and protein aggregation associated with human disease.…”
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Roles for Hsp40 molecular chaperones in protein misfolding disease

Table of Contents: “…Contents: Protein aggregation in conformational disease -- The Hsp40 system -- The Hsp70 system -- Small oligomers, prion, yeast, Huntington's disease and protein triage -- Mechanisms for proteotoxicity -- Mechanisms for amyloid disease.…”
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Clinical neuropathology text and color atlas / by Haberland, Catherine

Table of Contents: “…Basics of neuropathology -- Cerebral hypoxia -- Cerebrovascular diseases -- Neurodegenerative diseases -- Infectious diseases -- Prion diseases -- Demyelinating diseases -- Hereditary neurometabolic diseases -- Acquired neurometabolic diseases -- Tumors of the central nervous system -- Traumatic injuries of the central nervous system -- Congenital malformations of the central nervous system -- Fetal and perinatal cerebral pathology.…”
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Molecular Neurodegeneration

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Innate immunity in the brain in health and disease

Table of Contents: “…Contents: Macrophage populations in the brain -- Regulation of brain macrophage-microglia phenotype -- Inflammation in Alzheimer's disease -- Prion disease, a model of chronic neurodegeneration -- Behavioural assays -- Impact of systemic infections/inflammation on the brain -- Interactions between systemic inflammation and brain disease -- Microglia switching -- Relevance to Alzheimer's disease? …”
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The Nobel Prize winning discoveries in infectious diseases by Rifkind, David

Table of Contents: “…Serotherapy -- Antimicrobial defenses -- MHC restriction -- Prontosil and the sulfonamides -- Penicillin -- Streptomycin -- Chemotherapeutic agents -- Tuberculosis -- Typhus -- Syphilis therapy -- Tobacco mosaic virus -- Yellow fever -- Poliomyelitis virus -- Hepatitis B virus -- Bacteriophage -- Bacteriophage lysogeny -- Rous sarcoma virus -- Polyoma virus -- Reverse transcriptase -- Viral oncogenes -- Kuru -- Prions -- Malaria -- Cancer parasite -- DDT.…”
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