Challenges and Advances in Antemortem Diagnosis of Human Transmissible Spongiform Encephalopathies

Frontiers in Bioengineering and Biotechnology
Lucas M AscariYraima Cordeiro

Abstract

Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, arise from the structural conversion of the monomeric, cellular prion protein (PrPC) into its multimeric scrapie form (PrPSc). These pathologies comprise a group of intractable, rapidly evolving neurodegenerative diseases. Currently, a definitive diagnosis of TSE relies on the detection of PrPSc and/or the identification of pathognomonic histological features in brain tissue samples, which are usually obtained postmortem or, in rare cases, by brain biopsy (antemortem). Over the past two decades, several paraclinical tests for antemortem diagnosis have been developed to preclude the need for brain samples. Some of these alternative methods have been validated and can provide a probable diagnosis when combined with clinical evaluation. Paraclinical tests include in vitro cell-free conversion techniques, such as the real-time quaking-induced conversion (RT-QuIC), as well as immunoassays, electroencephalography (EEG), and brain bioimaging methods, such as magnetic resonance imaging (MRI), whose importance has increased over the years. PrPSc is the main biomarker in TSEs, and the RT-QuIC assay stands out for its ability to detect PrPSc in cerebrospinal f...Continue Reading

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Citations

May 1, 2021·Viruses·Mark P Figgie, Brian S Appleby
Jul 3, 2021·Special Care in Dentistry : Official Publication of the American Association of Hospital Dentists, the Academy of Dentistry for the Handicapped, and the American Society for Geriatric Dentistry·Mario Eduardo BaldoPaulo Sérgio da Silva Santos

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Methods Mentioned

BETA
glycosylation
nuclear magnetic resonance
biopsies
biopsy
transgenic
immunoprecipitation
ELISA
enzyme-linked immunosorbent assay
genotyping

Software Mentioned

PMCA
QuIC
RT

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