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Mostrando 201 - 220 Resultados de 4,339 Para Buscar '"Prion"', tiempo de consulta: 0.25s Limitar resultados
  1. 201
    “…Human familial prion diseases are associated with mutations at 34 different prion protein (PrP) amino acid residues. …”
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    Familial human prion diseases associated with prion protein mutations Y226X and G131V are transmissible to transgenic mice expressing human prion protein
  2. 202
    “…Prion diseases are fatal neurodegenerative disorders in which the main pathogenic event is the conversion of the cellular prion protein (PrP(C)) into an abnormal and misfolded isoform known as PrP(Sc). …”
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    Novel polymorphisms in the prion protein gene (PRNP) and stability of the resultant prion protein in different horse breeds
  3. 203
    “…The bank vole is a rodent susceptible to different prion strains from humans and various animal species. …”
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    Prion Protein Amino Acid Determinants of Differential Susceptibility and Molecular Feature of Prion Strains in Mice and Voles
  4. 204
    “…Converging evidence leaves little doubt that a change in the conformation of prion protein (PrP(C)) from a mainly α-helical to a β-sheet rich PrP-scrapie (PrP(Sc)) form is the main event responsible for prion disease associated neurotoxicity. …”
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    Prion Protein Modulates Cellular Iron Uptake: A Novel Function with Implications for Prion Disease Pathogenesis
  5. 205
    “…BACKGROUND: The propagation of prions, the causative agents of Creutzfeldt-Jakob disease and other human prion diseases, requires post-translational conversion of normal cellular prion protein to disease-associated forms. …”
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    Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient-preference trial
  6. 206
    “…In both vCJD and experimental BSE models prion agents do reach the bloodstream, raising concerns regarding disease transmission through blood transfusion. …”
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    Prion Protein Expression and Processing in Human Mononuclear Cells: The Impact of the Codon 129 Prion Gene Polymorphism
  7. 207
    “…Approximately 15 % of human prion disease is associated with autosomal-dominant pathogenic mutations in the prion protein (PrP) gene. …”
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    Absence of spontaneous disease and comparative prion susceptibility of transgenic mice expressing mutant human prion proteins
  8. 208
    “…Mammalian species vary widely in their apparent susceptibility to prion diseases. For example, several felid species developed prion disease (feline spongiform encephalopathy or FSE) during the bovine spongiform encephalopathy (BSE) epidemic in the United Kingdom, whereas no canine BSE cases were detected. …”
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    Genetic Predictions of Prion Disease Susceptibility in Carnivore Species Based on Variability of the Prion Gene Coding Region
  9. 209
    “…BACKGROUND: It has been widely established that the conversion of the cellular prion protein (PrP(C)) into its abnormal isoform (PrP(Sc)) is responsible for the development of transmissible spongiform encephalopathies (TSEs). …”
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    Peroxiredoxin 6 promotes upregulation of the prion protein (PrP) in neuronal cells of prion-infected mice
  10. 210
    “…Prions are proteinaceous infectious agents responsible for fatal neurodegenerative diseases in animals and humans. …”
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    Quaternary Structure of Pathological Prion Protein as a Determining Factor of Strain-Specific Prion Replication Dynamics
  11. 211
    “…Public and animal health controls to limit human exposure to animal prions are focused on bovine spongiform encephalopathy (BSE), but other prion strains in ruminants may also have zoonotic potential. …”
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    Atypical Scrapie Prions from Sheep and Lack of Disease in Transgenic Mice Overexpressing Human Prion Protein
  12. 212
    “…BACKGROUND: In humans and animals, prion protein (PrP) is usually expressed as a glycophosphatidylinositol (GPI)-anchored membrane protein, but anchorless PrP may be pathogenic in humans with certain familial prion diseases. …”
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    Distinct patterns of spread of prion infection in brains of mice expressing anchorless or anchored forms of prion protein
  13. 213
    “…These results suggest that a new therapeutic scheme based an innovative delivery system of PrP-siRNA can be envisioned in prion disorders.…”
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    Systemic Delivery of siRNA Down Regulates Brain Prion Protein and Ameliorates Neuropathology in Prion Disorder
  14. 214
    “…Prion diseases are neurodegenerative disorders characterized by the aggregation of abnormally folded prion protein (PrP(Sc)). …”
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    Increased expression of p62/SQSTM1 in prion diseases and its association with pathogenic prion protein
  15. 215
    “…BACKGROUND: Sporadic, inherited and acquired prion diseases show distinct histological patterns of abnormal prion protein (PrP) deposits. …”
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    Filamentous white matter prion protein deposition is a distinctive feature of multiple inherited prion diseases
  16. 216
    “…Prions are lethal infectious agents thought to consist of multi-chain forms (PrP(Sc)) of misfolded cellular prion protein (PrP(C)). …”
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    Prion neuropathology follows the accumulation of alternate prion protein isoforms after infective titre has peaked
  17. 217
    “…Misfolded isoform of prion protein (PrP), termed scrapie PrP (PrP(Sc)), tends to aggregate into various fibril forms. …”
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    Evaluation of Infective Property of Recombinant Prion Protein Amyloids in Cultured Cells Overexpressing Cellular Prion Protein
  18. 218
    “…Natural prion diseases of ruminants are moderately contagious and while the gastrointestinal tract is the primary site of prion agent entry, other mucosae may be entry sites in a subset of infections. …”
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    Lesion of the Olfactory Epithelium Accelerates Prion Neuroinvasion and Disease Onset when Prion Replication Is Restricted to Neurons
  19. 219
    “…In prion diseases, synapse dysfunction, axon retraction and loss of neuronal polarity precede neuronal death. …”
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    Double-Edge Sword of Sustained ROCK Activation in Prion Diseases through Neuritogenesis Defects and Prion Accumulation
  20. 220
    por Daus, Martin L.
    Publicado 2016
    “…In 1982, the term “prions” (proteinaceous infectious particles) was coined to specify a new principle of infection. …”
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    Disease Transmission by Misfolded Prion-Protein Isoforms, Prion-Like Amyloids, Functional Amyloids and the Central Dogma
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