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2018-06

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cc-by (c) Kanata, Eirini et al., 2018
Please use this identifier to cite or link to this item: https://hdl.handle.net/2445/140561

MicroRNA alterations in the brain and body fluids of human and animal príon disease models: current status and perspectives

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https://doi.org/10.3389/fnagi.2018.00220

Abstract

Prion diseases are transmissible progressive neurodegenerative conditions characterized by rapid neuronal loss accompanied by a heterogeneous neuropathology, including spongiform degeneration, gliosis and protein aggregation. The pathogenic mechanisms and the origins of prion diseases remain unclear on the molecular level. Even though neurodegenerative diseases, including prion diseases, represent distinct entities, their pathogenesis shares a number of features including disturbed protein homeostasis, an overload of protein clearance pathways, the aggregation of pathological altered proteins, and the dysfunction and/or loss of specific neuronal populations. Recently, direct links have been established between neurodegenerative diseases and miRNA dysregulated patterns. miRNAs are a class of small non-coding RNAs involved in the fundamental post-transcriptional regulation of gene expression. Studies of miRNA alterations in the brain and body fluids in human prion diseases provide important insights into potential miRNA-associated disease mechanisms and biomarker candidates. miRNA alterations in prion disease models represent a unique tool to investigate the cause-consequence relationships of miRNA dysregulation in prion disease pathology, and to evaluate the use of miRNAs in diagnosis as biomarkers. Here, we provide an overview of studies on miRNA alterations in human prion diseases and relevant disease models, in relation to pertinent studies on other neurodegenerative diseases. KEYWORDS: CSF; blood; brain; microRNAs; neurodegenerative disorders; prion diseases; prion diseases animal models; sCJD

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RNA, Malalties per prions, Models animals en la investigació, Cervell, Sang, Malalties neurodegeneratives

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RNA, Prion diseases, Animal models in research, Brain, Blood, Neurodegenerative Diseases

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Articles publicats en revistes (Patologia i Terapèutica Experimental)
Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))

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KANATA, Eirini, et al. MicroRNA alterations in the brain and body fluids of human and animal príon disease models: current status and perspectives. Frontiers in Aging Neuroscience. 2018. Vol. 10, num. e220. ISSN 1663-4365. [consulted: 13 of August of 2026]. Available at: https://hdl.handle.net/2445/140561

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