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Notch Signaling Pathway Is Activated in Motoneurons of Spinal Muscular Atrophy

Cellular Neurobiology Group, IUNICS and Department of Biology, University of the Balearic Islands, Palma de Mallorca 07122, Spain
Neuronal Signaling Unit, Department of Basic Medical Sciences, University of Lleida-IRBLLEIDA, Lleida 25198, Spain
Department of Medical Physiology and Biophysics, School of Medicine, University of Seville, Seville 41009, Spain
Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2013, 14(6), 11424-11437;
Received: 19 April 2013 / Revised: 2 May 2013 / Accepted: 17 May 2013 / Published: 29 May 2013
(This article belongs to the Section Biochemistry)
PDF [3895 KB, uploaded 19 June 2014]


Spinal muscular atrophy (SMA) is a neurodegenerative disease produced by low levels of Survival Motor Neuron (SMN) protein that affects alpha motoneurons in the spinal cord. Notch signaling is a cell-cell communication system well known as a master regulator of neural development, but also with important roles in the adult central nervous system. Aberrant Notch function is associated with several developmental neurological disorders; however, the potential implication of the Notch pathway in SMA pathogenesis has not been studied yet. We report here that SMN deficiency, induced in the astroglioma cell line U87MG after lentiviral transduction with a shSMN construct, was associated with an increase in the expression of the main components of Notch signaling pathway, namely its ligands, Jagged1 and Delta1, the Notch receptor and its active intracellular form (NICD). In the SMNΔ7 mouse model of SMA we also found increased astrocyte processes positive for Jagged1 and Delta1 in intimate contact with lumbar spinal cord motoneurons. In these motoneurons an increased Notch signaling was found, as denoted by increased NICD levels and reduced expression of the proneural gene neurogenin 3, whose transcription is negatively regulated by Notch. Together, these findings may be relevant to understand some pathologic attributes of SMA motoneurons. View Full-Text
Keywords: SMA; Notch; NICD; Jagged1; Delta1; Neurogenin 3; astrocyte; motoneuron SMA; Notch; NICD; Jagged1; Delta1; Neurogenin 3; astrocyte; motoneuron
This is an open access article distributed under the Creative Commons Attribution License (CC BY 3.0).

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Caraballo-Miralles, V.; Cardona-Rossinyol, A.; Garcera, A.; Torres-Benito, L.; Soler, R.M.; Tabares, L.; Lladó, J.; Olmos, G. Notch Signaling Pathway Is Activated in Motoneurons of Spinal Muscular Atrophy. Int. J. Mol. Sci. 2013, 14, 11424-11437.

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