Abstract
Prion diseases are fatal neurological disorders affecting various mammalian species including humans. Lack of proper diagnostic tools and non-availability of therapeutic remedies are hindering the control strategies for prion diseases. MicroRNAs (miRNAs) are abundant endogenous short non-coding essential RNA molecules that negatively regulate the target genes after transcription. Several biological processes depend on miRNAs, and altered profiles of these miRNAs are potential biomarkers for various neurodegenerative diseases, including prion diseases. Autophagic flux degrades the misfolded prion proteins to reduce chronic endoplasmic reticulum stress and enhance cell survival. Recent evidence suggests that specific miRNAs target and regulate the autophagic mechanism, which is critical for alleviating cellular stress. miRNAs-mediated regulation of these specific proteins involved in the autophagy represents a new target with highly significant therapeutic prospects. Here, we will briefly describe the biology of miRNAs, the use of miRNAs as potential biomarkers with their credibility, the regulatory mechanism of miRNAs in major neurodegenerative diseases such as Alzheimer’s, Parkinson’s, and prion diseases, degradation pathways for aggregated prion proteins, the role of autophagy in prion diseases. Finally, we will discuss the miRNAs-modulated autophagic flux in neurodegenerative diseases and employ them as potential therapeutic intervention strategy in prion diseases.
Highlights
Prion diseases known as transmissible spongiform encephalopathies (TSEs) are a family of neurodegenerative diseases affecting several mammalian species including humans
Many research groups focus on prion diseases since the discovery of a fatal prion disease in humans known as variant Creutzfeldt-Jakob disease (CJD) (Somerville et al, 1997; Lezmi et al, 2003)
Molecular research increasing the lifespan in different animal models is a promising start toward finding the best possible therapeutic intervention strategy in prion diseases
Summary
Prion diseases known as transmissible spongiform encephalopathies (TSEs) are a family of neurodegenerative diseases affecting several mammalian species including humans. MiRNA-342-3p was upregulated in scrapie-infected mice brains (Saba et al, 2008; Montag et al, 2009) These studies reveal that miRNA-342-3p might be a potential biomarker in the terminal stage of prion diseases. Studies involving prion-infected mouse brain showed that various gene promoters are related to miRNAs. Some important gene promoters identified were E2F-1 (cell cycle re-entry and neurodegeneration), MAZ (inflammatory response transcription factor), PAX6 (neurogenesis), KROX (transcription factor), and early growth response 1 (EGR1). Gibbings et al (2012) demonstrated that autophagy selectively degrades miRNA-processing enzyme, Dicer and the main miRNA effector protein, Ago, by selective autophagy receptor NDP52, regulating miRNAs biogenesis This shows that NDP52 and autophagy plays a crucial role in the homeostasis and activity of miRNAs (Gibbings et al, 2012). Substantia nigra pars Compacta, Substantia nigra pars Compacta, 76 Amygdala 43 Alzheimer’s disease Blood 32, 10 Blood 7 Peripheral Blood 215
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