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Int. J. Mol. Sci. 2015, 16(8), 18312-18327; doi:10.3390/ijms160818312

A Perturbed MicroRNA Expression Pattern Characterizes Embryonic Neural Stem Cells Derived from a Severe Mouse Model of Spinal Muscular Atrophy (SMA)

1
Department of Biomedicine and Prevention, University of Rome Tor Vergata, Via Montpellier 1, 00133 Rome, Italy
2
Gene Expression-Microarrays Laboratory, Bambino Gesù Children's Hospital-IRCCS Polo di Ricerca-V.le di San Paolo 15, 00146 Rome, Italy
3
Department of Cell Biology and Neurosciences, Istituto Superiore di Sanità, 00161 Rome, Italy
*
Author to whom correspondence should be addressed.
Academic Editor: Nalini Santanam
Received: 8 July 2015 / Revised: 27 July 2015 / Accepted: 31 July 2015 / Published: 6 August 2015
(This article belongs to the Special Issue MicroRNA in Various Disease States as Biomarkers)
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Abstract

Spinal muscular atrophy (SMA) is an inherited neuromuscular disorder and the leading genetic cause of death in infants. Despite the disease-causing gene, survival motor neuron (SMN1), encodes a ubiquitous protein, SMN1 deficiency preferentially affects spinal motor neurons (MNs), leaving the basis of this selective cell damage still unexplained. As neural stem cells (NSCs) are multipotent self-renewing cells that can differentiate into neurons, they represent an in vitro model for elucidating the pathogenetic mechanism of neurodegenerative diseases such as SMA. Here we characterize for the first time neural stem cells (NSCs) derived from embryonic spinal cords of a severe SMNΔ7 SMA mouse model. SMNΔ7 NSCs behave as their wild type (WT) counterparts, when we consider neurosphere formation ability and the expression levels of specific regional and self-renewal markers. However, they show a perturbed cell cycle phase distribution and an increased proliferation rate compared to wild type cells. Moreover, SMNΔ7 NSCs are characterized by the differential expression of a limited number of miRNAs, among which miR-335-5p and miR-100-5p, reduced in SMNΔ7 NSCs compared to WT cells. We suggest that such miRNAs may be related to the proliferation differences characterizing SMNΔ7 NSCs, and may be potentially involved in the molecular mechanisms of SMA. View Full-Text
Keywords: survival motor neuron (SMN); spinal muscular atrophy (SMA); neural stem cells (NSCs); motor neurons (MNs); microRNAs (miRNAs) survival motor neuron (SMN); spinal muscular atrophy (SMA); neural stem cells (NSCs); motor neurons (MNs); microRNAs (miRNAs)
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Luchetti, A.; Ciafrè, S.A.; Murdocca, M.; Malgieri, A.; Masotti, A.; Sanchez, M.; Farace, M.G.; Novelli, G.; Sangiuolo, F. A Perturbed MicroRNA Expression Pattern Characterizes Embryonic Neural Stem Cells Derived from a Severe Mouse Model of Spinal Muscular Atrophy (SMA). Int. J. Mol. Sci. 2015, 16, 18312-18327.

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