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Prion diseases: immunotargets and therapy

Overview of attention for article published in ImmunoTargets and Therapy, June 2016
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93 Mendeley
Title
Prion diseases: immunotargets and therapy
Published in
ImmunoTargets and Therapy, June 2016
DOI 10.2147/itt.s64795
Pubmed ID
Authors

Peter Panegyres, Jennifer T. Burchell

Abstract

Transmissible spongiform encephathalopathies or prion diseases are a group of neurological disorders characterized by neuronal loss, spongiform degeneration, and activation of astrocytes or microglia. These diseases affect humans and animals with an extremely high prevalence in some species such as deer and elk in North America. Although rare in humans, they result in a devastatingly swift neurological progression with dementia and ataxia. Patients usually die within a year of diagnosis. Prion diseases are familial, sporadic, iatrogenic, or transmissible. Human prion diseases include Kuru, sporadic, iatrogenic, and familial forms of Creutzfeldt-Jakob disease, variant Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia. The causative agent is a misfolded version of the physiological prion protein called PrP(Sc) in the brain. There are a number of therapeutic options currently under investigation. A number of small molecules have had some success in delaying disease progression in animal models and mixed results in clinical trials, including pentosan polysulfate, quinacrine, and amphotericin B. More promisingly, immunotherapy has reported success in vitro and in vivo in animal studies and clinical trials. The three main branches of immunotherapy research are focus on antibody vaccines, dendritic cell vaccines, and adoptive transfer of physiological prion protein-specific CD4(+) T-lymphocytes. Vaccines utilizing antibodies generally target disease-specific epitopes that are only exposed in the misfolded PrP(Sc) conformation. Vaccines utilizing antigen-loaded dendritic cell have the ability to bypass immune tolerance and prime CD4(+) cells to initiate an immune response. Adoptive transfer of CD4(+) T-cells is another promising target as this cell type can orchestrate the adaptive immune response. Although more research into mechanisms and safety is required, these immunotherapies offer novel therapeutic targets for prion diseases.

Twitter Demographics

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Mendeley readers

The data shown below were compiled from readership statistics for 93 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Colombia 1 1%
Unknown 92 99%

Demographic breakdown

Readers by professional status Count As %
Student > Bachelor 29 31%
Student > Master 15 16%
Student > Doctoral Student 9 10%
Researcher 7 8%
Student > Ph. D. Student 6 6%
Other 9 10%
Unknown 18 19%
Readers by discipline Count As %
Biochemistry, Genetics and Molecular Biology 26 28%
Medicine and Dentistry 17 18%
Agricultural and Biological Sciences 8 9%
Neuroscience 7 8%
Immunology and Microbiology 3 3%
Other 12 13%
Unknown 20 22%