UCL Discovery
UCL home » Library Services » Electronic resources » UCL Discovery

Review: Fluid biomarkers in the human prion diseases

Thompson, AGB; Mead, SH; (2019) Review: Fluid biomarkers in the human prion diseases. Molecular and Cellular Neuroscience , 97 pp. 81-92. 10.1016/j.mcn.2018.12.003. Green open access

[thumbnail of 1-s2.0-S1044743118303415-main.pdf]
Preview
Text
1-s2.0-S1044743118303415-main.pdf - Accepted Version

Download (1MB) | Preview

Abstract

The human prion diseases are a diverse set of often rapidly progressive neurodegenerative conditions associated with abnormal forms of the prion protein. We review work to establish diagnostic biomarkers and assays that might fill other important roles, particularly those that could assist the planning and interpretation of clinical trials. The field now benefits from highly sensitive and specific diagnostic biomarkers using cerebrospinal fluid: detecting by-products of rapid neurodegeneration or specific functional properties of abnormal prion protein, with the second generation real time quaking induced conversion (RT-QuIC) assay being particularly promising. Blood has been a more challenging analyte, but has now also yielded valuable biomarkers. Blood-based assays have been developed with the potential to screen for variant Creutzfeldt-Jakob disease, although it remains uncertain whether these will ever be used in practice. The very rapid neurodegeneration of prion disease results in strong signals from surrogate protein markers in the blood that reflect neuronal, axonal, synaptic or glial pathology in the brain: notably the tau and neurofilament light chain proteins. We discuss early evidence that such tests, applied alongside robust diagnostic biomarkers, may have potential to add value as clinical trial outcome measures, predictors of future disease course (including for asymptomatic individuals at high risk of prion disease), and as rapidly accessible and sensitive markers to aid early diagnosis.

Type: Article
Title: Review: Fluid biomarkers in the human prion diseases
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.mcn.2018.12.003
Publisher version: https://doi.org/10.1016/j.mcn.2018.12.003
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Biomarker, Blood, CJD, CSF, NFL, Prion, RT-QuIC, Tau
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Institute of Prion Diseases
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Institute of Prion Diseases > MRC Prion Unit at UCL
URI: https://discovery.ucl.ac.uk/id/eprint/10064537
Downloads since deposit
221Downloads
Download activity - last month
Download activity - last 12 months
Downloads by country - last 12 months

Archive Staff Only

View Item View Item