The prion hypothesis: from biological anomaly to basic regulatory mechanism

Prions

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Novel Proteinaceous Infectious Particles Cause Scrapie

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Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent

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Lewy bodies in grafted neurons in subjects with Parkinson's disease suggest host-to-graft disease propagation

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Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD

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Curli biogenesis and function.

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[URE3] as an Altered URE2 Protein: Evidence for a Prion Analog in Saccharomyces cerevisiae

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Nature of the Scrapie Agent: Self-replication and Scrapie

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A General Model of Prion Strains and Their Pathogenicity

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Functional Amyloids As Natural Storage of Peptide Hormones in Pituitary Secretory Granules

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Exogenous Induction of Cerebral ß-Amyloidogenesis Is Governed by Agent and Host

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A Systematic Survey Identifies Prions and Illuminates Sequence Features of Prionogenic Proteins

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Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication

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Functional Amyloid Formation within Mammalian Tissue

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Normal host prion protein necessary for scrapie-induced neurotoxicity

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Prion-like transmission of protein aggregates in neurodegenerative diseases

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Depleting Neuronal PrP in Prion Infection Prevents Disease and Reverses Spongiosis

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A yeast prion provides a mechanism for genetic variation and phenotypic diversity

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Anchorless Prion Protein Results in Infectious Amyloid Disease Without Clinical Scrapie

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Support for the Prion Hypothesis for Inheritance of a Phenotypic Trait in Yeast

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The physical basis of how prion conformations determine strain phenotypes

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Common mechanisms of amyloid oligomer pathogenesis in degenerative disease

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A Neuronal Isoform of the Aplysia CPEB Has Prion-Like Properties

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Prions Affect the Appearance of Other Prions

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Expression of Amino-Terminally Truncated PrP in the Mouse Leading to Ataxia and Specific Cerebellar Lesions

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Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation

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Ψ, A cytoplasmic suppressor of super-suppressor in yeast

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Slow transmissible diseases of the nervous system

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Propagation of the yeast prion‐like [psi+] determinant is mediated by oligomerization of the SUP35‐encoded polypeptide chain release factor.

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Rnq1

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Distinct prion proteins in short and long scrapie incubation period mice

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A census of glutamine/asparagine-rich regions: Implications for their conserved function and the prediction of novel prions

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The protein product of the het-s heterokaryon incompatibility gene of the fungus Podospora anserina behaves as a prion analog

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Pathway Complexity of Prion Protein Assembly into Amyloid

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Darwinian Evolution of Prions in Cell Culture

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The cellular prion protein (PrPC): Its physiological function and role in disease

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PrP Genotype and Agent Effects in Scrapie: Change In Allelic Interaction With Different Isolates of Agent in Sheep, a Natural Host of Scrapie

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The Prion's Elusive Reason for Being

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Cytoplasmic penetration and persistent infection of mammalian cells by polyglutamine aggregates

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Axonal prion protein is required for peripheral myelin maintenance

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Aplysia CPEB Can Form Prion-like Multimers in Sensory Neurons that Contribute to Long-Term Facilitation

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Growth control and ribosome biogenesis

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A Critical Role for Amino-Terminal Glutamine/Asparagine Repeats in the Formation and Propagation of a Yeast Prion

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Molecular recycling within amyloid fibrils

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Epigenetic regulation of translation reveals hidden genetic variation to produce complex traits

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Emerging Principles of Conformation-Based Prion Inheritance

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Influence of Amino Acid Substitutions Related to Inherited Human Prion Diseases on the Thermodynamic Stability of the Cellular Prion Protein

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Multiple Gln/Asn-Rich Prion Domains Confer Susceptibility to Induction of the Yeast [PSI] Prion

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Species-barrier-independent prion replication in apparently resistant species

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Proprotein convertase cleavage liberates a fibrillogenic fragment of a resident glycoprotein to initiate melanosome biogenesis

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

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Targeting Cellular Prion Protein Reverses Early Cognitive Deficits and Neurophysiological Dysfunction in Prion-Infected Mice

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PrP gene dosage determines the timing but not the final intensity or distribution of lesions in scrapie pathology.

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The yeast global transcriptional co-repressor protein Cyc8 can propagate as a prion

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Fibril Conformation as the Basis of Species- and Strain-Dependent Seeding Specificity of Mammalian Prion Amyloids

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Evidence that the Transmission of One Source of Scrapie Agent to Hamsters Involves Separation of Agent Strains from a Mixture

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Yeast prions [URE3] and [PSI + ] are diseases

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Interactions among prions and prion “strains” in yeast

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High Prion and PrPSc Levels but Delayed Onset of Disease in Scrapie-Inoculated Mice Heterozygous for a Disrupted PrP Gene

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Quantifying the kinetic parameters of prion replication

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Prion diseases of humans and animals

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Prion Strain Mutation Determined by Prion Protein Conformational Compatibility and Primary Structure

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Slow Virus Diseases of Animals and Man

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Prions of fungi: inherited structures and biological roles

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Lethal recessive myelin toxicity of prion protein lacking its central domain

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Regulation of Embryonic Cell Adhesion by the Prion Protein

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Continuum of prion protein structures enciphers a multitude of prion isolate-specified phenotypes

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Translation termination efficiency can be regulated in Saccharomyces cerevisiae by environmental stress through a prion‐mediated mechanism

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Effects of Q/N-rich, polyQ, and non-polyQ amyloids on the de novo formation of the [ PSI + ] prion in yeast and aggregation of Sup35 in vitro

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Primary sequence independence for prion formation

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Single treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease

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The dominant PNM2- mutation which eliminates the psi factor of Saccharomyces cerevisiae is the result of a missense mutation in the SUP35 gene.

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Dependence and independence of [PSI+] and [PIN+]: a two‐prion system in yeast?

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Dissection and Design of Yeast Prions

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Beyond the prion principle

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Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt–Jakob disease: its effect on the phenotype and prion-type characteristics

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The prion strain phenomenon: Molecular basis and unprecedented features

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Codon 219 lys allele of PRNP is not found in sporadic Creutzfeldt‐Jakob disease

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The Prion Protein Knockout Mouse

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Continuous Quinacrine Treatment Results in the Formation of Drug-Resistant Prions

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Dominant-negative inhibition of prion replication in transgenic mice

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Adaptation and Selection of Prion Protein Strain Conformations following Interspecies Transmission of Transmissible Mink Encephalopathy

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Fungal incompatibility: Evolutionary origin in pathogen defense?

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