Prion diseases and their biochemical mechanisms.

No abstract is available for this paper.

• Publication year

  2009

• Venue

  Biochemistry

• Publication date

  2009-03-31

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1021/bi900108vPMID 19239250PMCID PMC2805067
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

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• Role of Erk1/2 activation in prion disease pathogenesis: absence of CCR1 leads to increased Erk1/2 activation and accelerated disease progression.

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• Crossing the species barrier by PrP(Sc) replication in vitro generates unique infectious prions.

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• Prion Protein Amyloid Formation under Native-like Conditions Involves Refolding of the C-terminal α-Helical Domain*

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• Fecal transmission of AA amyloidosis in the cheetah contributes to high incidence of disease

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• Mechanisms of prion protein assembly into amyloid

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• The Same Primary Structure of the Prion Protein Yields Two Distinct Self-propagating States*

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• Protein inheritance (prions) based on parallel in‐register β‐sheet amyloid structures

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• Newly identified prion linked to the chromatin-remodeling factor Swi1 in Saccharomyces cerevisiae

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• Hippocampal bursts caused by changes in NMDA receptor-dependent excitation in a mouse model of variant CJD.

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• Prions and peripheral nerves: A deadly rendezvous

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• The cellular prion protein (PrP(C)): its physiological function and role in disease.

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• Formation of native prions from minimal components in vitro

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• Ultrasensitive detection of scrapie prion protein using seeded conversion of recombinant prion protein

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• Molecular architecture of human prion protein amyloid: A parallel, in-register β-structure

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• A General Model of Prion Strains and Their Pathogenicity

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• The physical basis of how prion conformations determine strain phenotypes

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• Early intermediate in human prion protein folding as evidenced by ultrarapid mixing experiments.

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• The stoichiometry of host PrPC glycoforms modulates the efficiency of PrPSc formation in vitro.

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• Interaction of metals with prion protein: possible role of divalent cations in the pathogenesis of prion diseases.

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• Structure of the cross-beta spine of amyloid-like fibrils.

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• Structure of the cross-β spine of amyloid-like fibrils

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• In vitro generation of infectious scrapie prions.

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• Anchorless Prion Protein Results in Infectious Amyloid Disease Without Clinical Scrapie

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• The most infectious prion protein particles

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• Immunodetection of disease‐associated mutant PrP, which accelerates disease in GSS transgenic mice

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• Synthetic prions generated in vitro are similar to a newly identified subpopulation of PrPSc from sporadic Creutzfeldt‐Jakob Disease

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• Mechanism of cross-species prion transmission: an infectious conformation compatible with two highly divergent yeast prion proteins.

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• Fibril conformation as the basis of species- and strain-dependent seeding specificity of mammalian prion amyloids.

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• From conversion to aggregation: protofibril formation of the prion protein.

  2004cited by this paper
• Evidence for assembly of prions with left-handed β-helices into trimers

  2004cited by this paper
• The Effect of Disease-associated Mutations on the Folding Pathway of Human Prion Protein*

  2004cited by this paper
• Evidence for assembly of prions with left-handed beta-helices into trimers.

  2004cited by this paper
• Copper binding in the prion protein.

  2004cited by this paper
• NMR structure of the bovine prion protein isolated from healthy calf brains

  2004cited by this paper
• The state of the prion

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• Conformational variations in an infectious protein determine prion strain differences

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• Protein-only transmission of three yeast prion strains

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• Synthetic Mammalian Prions

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• Prions: proteins as genes and infectious entities.

  2004cited by this paper
• Autocatalytic Conversion of Recombinant Prion Proteins Displays a Species Barrier*

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• Molecular basis of barriers for interspecies transmissibility of mammalian prions.

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• Mammalian prion biology: one century of evolving concepts.

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• Mechanism of Prion Propagation: Amyloid Growth Occurs by Monomer Addition

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• Protease-Resistant Human Prion Protein and Ferritin Are Cotransported across Caco-2 Epithelial Cells: Implications for Species Barrier in Prion Uptake from the Intestine

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• Progress towards a molecular-level structural understanding of amyloid fibrils.

  2004cited by this paper
• Seeded conversion of recombinant prion protein to a disulfide-bonded oligomer by a reduction-oxidation process

  2003cited by this paper
• Molecular Distinction between Pathogenic and Infectious Properties of the Prion Protein

  2003cited by this paper
• Protofibrils, pores, fibrils, and neurodegeneration: separating the responsible protein aggregates from the innocent bystanders.

  2003cited by this paper
• Crystal structure of the human prion protein reveals a mechanism for oligomerization

  2002cited by this paper
• Exogenous induction of cerebral β-amyloidosis in βAPP-transgenic mice

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• Islet amyloid: phase partitioning and secondary nucleation are central to the mechanism of fibrillogenesis.

  2002cited by this paper
• Differences between the prion protein and its homolog Doppel: a partially structured state with implications for scrapie formation.

  2002cited by this paper
• Locally disordered conformer of the hamster prion protein: a crucial intermediate to PrPSc?

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• Prions as protein-based genetic elements.

  2002cited by this paper
• Exogenous induction of cerebral beta-amyloidosis in betaAPP-transgenic mice.

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• Kinetic Intermediate in the Folding of Human Prion Protein*

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• A change in the conformation of prions accompanies the emergence of a new prion strain.

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• Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding

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• The Role of Disulfide Bridge in the Folding and Stability of the Recombinant Human Prion Protein*

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  1999cited by this paper
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  1991cited by this paper
• Acquisition of protease resistance by prion proteins in scrapie-infected cells does not require asparagine-linked glycosylation.

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  1990cited by this paper
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  1989cited by this paper
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