Analysis of the fibroblast growth factor system reveals alterations in a mouse model of spinal muscular atrophy.

PloS One
Niko HenselPeter Claus

Abstract

The monogenetic disease Spinal Muscular Atrophy (SMA) is characterized by a progressive loss of motoneurons leading to muscle weakness and atrophy due to severe reduction of the Survival of Motoneuron (SMN) protein. Several models of SMA show deficits in neurite outgrowth and maintenance of neuromuscular junction (NMJ) structure. Survival of motoneurons, axonal outgrowth and formation of NMJ is controlled by neurotrophic factors such as the Fibroblast Growth Factor (FGF) system. Besides their classical role as extracellular ligands, some FGFs exert also intracellular functions controlling neuronal differentiation. We have previously shown that intracellular FGF-2 binds to SMN and regulates the number of a subtype of nuclear bodies which are reduced in SMA patients. In the light of these findings, we systematically analyzed the FGF-system comprising five canonical receptors and 22 ligands in a severe mouse model of SMA. In this study, we demonstrate widespread alterations of the FGF-system in both muscle and spinal cord. Importantly, FGF-receptor 1 is upregulated in spinal cord at a pre-symptomatic stage as well as in a mouse motoneuron-like cell-line NSC34 based model of SMA. Consistent with that, phosphorylations of FGFR-downs...Continue Reading

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Citations

Apr 9, 2013·PloS One·Dione T KobayashiKaren S Chen
Jun 13, 2013·Proceedings of the National Academy of Sciences of the United States of America·Anindya SenSpyros Artavanis-Tsakonas
Mar 17, 2017·Cell Death & Disease·Hee-Jun KimEun-Kyoung Choi
Apr 5, 2016·Journal of Experimental Neuroscience·Saif AhmadLaxman Gangwani
May 16, 2019·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Bianca Schröder-HeurichFrauke von Versen-Höynck
Jan 2, 2015·Cellular and Molecular Life Sciences : CMLS·Benjamin FörthmannPeter Claus
Apr 14, 2018·International Journal of Molecular Sciences·Hee-Jun KimEun-Kyoung Choi
Jan 6, 2021·International Journal of Molecular Sciences·Friederike Freiin von HövelClaudia Grothe
May 2, 2021·Proceedings of the National Academy of Sciences of the United States of America·Niko HenselPeter Claus
Aug 28, 2021·International Journal of Molecular Sciences·Li Chuin ChongSy-Bing Choi

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Methods Mentioned

BETA
reverse transcription PCR
SMA
GTPases
FCS
PCR

Software Mentioned

StepOne
GraphPad Prism
ImageJ
software

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