Translational Research in Serious Human Diseases

0536500 - ÚŽFG 2021 RIV CZ eng M - Monography Chapter
Valeková, Ivona - Motlík, Jan
Innate immunity in Huntington´s disease.
Translational Research in Serious Human Diseases. Praha: Academia, 2020 - (Kello, M.; Strnadel, J.; Klempir, J.; Roth, J.; Myslivcová-Fučíková, A.; Hansíková, H.; Kozák, I.), s. 49-56. ISBN 978-80-200-3158-7
R&D Projects: GA MŠMT(CZ) LO1609
Institutional support: RVO:67985904
Keywords : innate imunity * immune response * neuroinflammation
OECD category: Neurosciences (including psychophysiology

Huntington's disease (HD) is an inherited progressive neurodegenerative disorder with impairment of motor and cognitive functions. Any preventive or disease-modifying therapies are not available so far. Symptomatic treatment can only affect symptoms and it is not satisfying. Several approaches using RNAi to lower the expression of mutant huntingtin (mHTT) have been developed to date. In order to monitor the success of therapy efficacy in the pre-manifest stages of HD it is important to identify robust biomarkers of the onset and disease progression. The primary pathology of HD results inter alia from massive degeneration of neurons in the basal ganglia and thalamus. However, the expression of mutant huntingtin was detected in all examined tissues. Studies on HD demonstrated altered immune response in HD gene carriers indicating that cytokines may have a significant role in disease development. Therefore, cerebrospinal fluid (CSF) and blood serum may provide insight into pathology of HD, new prospective biomarkers and potential therapeutic targets. Additionally, microglia and monocytes could also serve as possible cellular source of candidate biomarkers to monitor HD progression.
Permanent Link: http://hdl.handle.net/11104/0314276