Prion diseases of humans and farm animals: epidemiology, genetics, and pathogenesis (Q36521947)

14 August 2017

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title

Prion diseases of humans and farm animals: epidemiology, genetics, and pathogenesis (English)

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14 August 2017

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Adriano Aguzzi

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language of work or name

English

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publication date

1 June 2006

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Journal of Neurochemistry

14 August 2017

published in

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14 August 2017

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14 August 2017

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14 August 2017

page(s)

1726-1739

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Understanding the diversity of prions

14 August 2017

cites work

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Dangerous liaisons between a microbe and the prion protein

21 January 2018

1 reference

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21 January 2018

A suspicious signature

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The exceptionally small size of the scrapie agent

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Diagnostic tests for small ruminant lentiviruses.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

PrP-expressing tissue required for transfer of scrapie infectivity from spleen to brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Natural and experimental oral infection of nonhuman primates by bovine spongiform encephalopathy agents

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Normal host prion protein necessary for scrapie-induced neurotoxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Normal host prion protein (PrPC) is required for scrapie spread within the central nervous system

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

"Friendly fire" in medicine: hormones, homografts, and Creutzfeldt-Jakob disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Role of microglia and host prion protein in neurotoxicity of a prion protein fragment

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Mice devoid of PrP are resistant to scrapie

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

In situ PCR-associated immunohistochemistry identifies cell types harbouring the Maedi-Visna virus genome in tissue sections of sheep infected naturally.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Cellular isoform of the scrapie agent protein participates in lymphocyte activation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

In vitro generation of infectious scrapie prions.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Encephalopathy in mice produced by inoculation with scrapie brain material

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Identification of scrapie prion protein-specific mRNA in scrapie-infected and uninfected brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Molecular distinction between pathogenic and infectious properties of the prion protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Prion protein is necessary for normal synaptic function

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Prion protein gene analysis in new variant cases of Creutzfeldt-Jakob disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

RNA molecules stimulate prion protein conversion

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Identification of a gene which controls the incubation period of some strains of scrapie agent in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Mutant PrP is delayed in its exit from the endoplasmic reticulum, but neither wild-type nor mutant PrP undergoes retrotranslocation prior to proteasomal degradation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Scrapie prion protein accumulation by scrapie-infected neuroblastoma cells abrogated by exposure to a prion protein antibody

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Sympathetic noradrenergic innervation of immune organs

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Binding of disease-associated prion protein to plasminogen.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Neurotoxicity of a prion protein fragment

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Immunoaffinity purification and neutralization of scrapie prion infectivity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Sympathetic innervation of lymphoreticular organs is rate limiting for prion neuroinvasion

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Incidence of Creutzfeldt-Jakob disease in Switzerland

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Human prion diseases: epidemiology and integrated risk assessment

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Transmissible familial Creutzfeldt-Jakob disease associated with five, seven, and eight extra octapeptide coding repeats in the PRNP gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

The sympathetic nervous system is involved in variant Creutzfeldt-Jakob disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

A transmembrane form of the prion protein in neurodegenerative disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Transmissible and genetic prion diseases share a common pathway of neurodegeneration

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Lymphoid follicle destruction and immunosuppression after repeated CpG oligodeoxynucleotide administration

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Chronic Lymphocytic Inflammation Specifies the Organ Tropism of Prions

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Post-translational import of the prion protein into the endoplasmic reticulum interferes with cell viability: a critical role for the putative transmembrane domain

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Prevention of scrapie pathogenesis by transgenic expression of anti-prion protein antibodies

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

The same prion strain causes vCJD and BSE

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Prevalence of lymphoreticular prion protein accumulation in UK tissue samples.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Specific binding of normal prion protein to the scrapie form via a localized domain initiates its conversion to the protease-resistant state

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Transmission of BSE by blood transfusion in sheep

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

HLA-DQ7 antigen and resistance to variant CJD.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Efficient lymphoreticular prion propagation requires PrP(c) in stromal and hematopoietic cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Protein-only transmission of three yeast prion strains

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

PrP expression in B lymphocytes is not required for prion neuroinvasion

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Complement facilitates early prion pathogenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Cell-free formation of protease-resistant prion protein.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Prion (PrPSc)-specific epitope defined by a monoclonal antibody

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Neurotoxicity of prion peptide 106-126 not confirmed

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Synthetic mammalian prions

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Strain-specified characteristics of mouse synthetic prions

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

PrPSc in mammary glands of sheep affected by scrapie and mastitis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Identification of multiple quantitative trait loci linked to prion disease incubation period in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Conversion of PrP to a self-perpetuating PrPSc-like conformation in the cytosol

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Neurotoxicity and neurodegeneration when PrP accumulates in the cytosol

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Temporary depletion of complement component C3 or genetic deficiency of C1q significantly delays onset of scrapie.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Temporary blockade of the tumor necrosis factor receptor signaling pathway impedes the spread of scrapie to the brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Follicular dendritic cell dedifferentiation by treatment with an inhibitor of the lymphotoxin pathway dramatically reduces scrapie susceptibility

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Genetic and environmental factors modify bovine spongiform encephalopathy incubation period in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

A protease-resistant protein is a structural component of the scrapie prion

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Soluble dimeric prion protein binds PrP(Sc) in vivo and antagonizes prion disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Impaired prion replication in spleens of mice lacking functional follicular dendritic cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Mice with gene targetted prion protein alterations show that Prnp, Sinc and Prni are congruent

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

A cellular gene encodes scrapie PrP 27-30 protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Conversion of alpha-helices into beta-sheets features in the formation of the scrapie prion proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

A prion protein epitope selective for the pathologically misfolded conformation

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

MHC typing in variant Creutzfeldt-Jakob disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Antibodies inhibit prion propagation and clear cell cultures of prion infectivity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Routes of transmission and consequences of small ruminant lentiviruses (SRLVs) infection and eradication schemes

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Humoral immune response to native eukaryotic prion protein correlates with anti-prion protection.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Colostral transmission of maedi visna virus: sites of viral entry in lambs born from experimentally infected ewes

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Prion pathogenesis in the absence of Toll-like receptor signalling

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Oral prion infection requires normal numbers of Peyer's patches but not of enteric lymphocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Positioning of follicular dendritic cells within the spleen controls prion neuroinvasion

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Molecular properties, partial purification, and assay by incubation period measurements of the hamster scrapie agent.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

NMR structure of the mouse prion protein domain PrP(121-231)

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Mimicry of variant Creutzfeldt-Jakob disease by sporadic Creutzfeldt-Jakob disease: importance of the pulvinar sign

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Cytosolic prion protein is not toxic and protects against Bax-mediated cell death in human primary neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Eight prion strains have PrP(Sc) molecules with different conformations

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

No propagation of prions in mice devoid of PrP.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Coincident scrapie infection and nephritis lead to urinary prion excretion

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Postexposure prophylaxis against prion disease with a stimulator of innate immunity

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Codon 219 Lys allele of PRNP is not found in sporadic Creutzfeldt-Jakob disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Modulation of proteinase-K resistant prion protein by prion peptide immunization.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Conformational variations in an infectious protein determine prion strain differences

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Estimation of epidemic size and incubation time based on age characteristics of vCJD in the United Kingdom

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Cellular prion protein promotes Brucella infection into macrophages

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

PrP knock-out and PrP transgenic mice in prion research

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

A novel progressive spongiform encephalopathy in cattle

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Distinct prion proteins in short and long scrapie incubation period mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

A new variant of Creutzfeldt-Jakob disease in the UK.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

CHRONIC WASTING DISEASE OF CAPTIVE MULE DEER: A SPONGIFORM ENCEPHALOPATHY

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Detection of pathologic prion protein in the olfactory epithelium in sporadic Creutzfeldt-Jakob disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Altered circadian activity rhythms and sleep in mice devoid of prion protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

Monoclonal antibodies inhibit prion replication and delay the development of prion disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1471-4159.2006.03909.X

10.1111/J.1471-4159.2006.03909.X

21 January 2018

Identifiers

DOI

1 reference

14 August 2017

1 reference