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The identity of muscle secreted factors critical for the development and maintenance of neuromuscular junctions (NMJs) remains largely unknown. Here, we show that muscle fibers secrete and concentrate the fibroblast growth factor binding protein 1 (FGFBP1) at NMJs. Although FGFBP1 expression increases during development, its expression decreases before NMJ degeneration during aging and in SOD1G93A mice, a mouse model for amyotrophic lateral sclerosis (ALS). Based on these findings, we examined the impact of deleting FGFBP1 on NMJs. In the absence of FGFBP1, NMJs exhibit structural abnormalities in developing and middle age mice. Deletion of FGFBP1 from SOD1G93A mice also accelerates NMJ degeneration and death. Based on these findings, we sought to identify the mechanism responsible for decreased FGFBP1 in stressed skeletal muscles. We show that FGFBP1 expression is inhibited by increased accumulation of the transforming growth factor-β1 (TGF-β1) in skeletal muscles and at their NMJs. These findings suggest that targeting the FGFBP1 and TGF-β1 signaling axis holds promise for slowing age- and disease-related degeneration of NMJs.
Pubmed ID: 28053031 RIS Download
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This unknown targets α-subunit of the nicotinic acetylcholine receptor (AChR) of neuromuscular junction
View all literature mentionsThis monoclonal targets myosin, sarcomere (MHC)
View all literature mentionsThis polyclonal targets Rabbit FGFBP
View all literature mentionsThis unknown targets zebrafish neuropil region and primary motor neuron axons
View all literature mentionsThis unknown targets NF
View all literature mentionsThis unknown targets Mouse IgG (H+L)
View all literature mentionsThis monoclonal targets Argonaute 2
View all literature mentionsCell line C2C12 is a Spontaneously immortalized cell line with a species of origin Mus musculus (Mouse)
View all literature mentionsMus musculus with name B6N.129-Rpl22tm1.1Psam/J from IMSR.
View all literature mentionsMus musculus with name B6.Cg-Tg(Thy1-YFP)16Jrs/J from IMSR.
View all literature mentionsMus musculus with name B6.129P2-Pvalbtm1(cre)Arbr/J from IMSR.
View all literature mentionsMus musculus with name B6.Cg-Tg(SOD1*G93A)1Gur/J from IMSR.
View all literature mentionsCell line Lex-2 is a Embryonic stem cell with a species of origin Mus musculus (Mouse)
View all literature mentionsThis monoclonal targets myosin, sarcomere (MHC)
View all literature mentionsThis polyclonal targets Rabbit FGFBP
View all literature mentionsThis unknown targets zebrafish neuropil region and primary motor neuron axons
View all literature mentionsThis unknown targets NF
View all literature mentionsThis unknown targets Mouse IgG (H+L)
View all literature mentionsThis monoclonal targets Argonaute 2
View all literature mentionsCell line C2C12 is a Spontaneously immortalized cell line with a species of origin Mus musculus (Mouse)
View all literature mentionsMus musculus with name B6N.129-Rpl22tm1.1Psam/J from IMSR.
View all literature mentionsMus musculus with name B6.Cg-Tg(Thy1-YFP)16Jrs/J from IMSR.
View all literature mentionsMus musculus with name B6.129P2-Pvalbtm1(cre)Arbr/J from IMSR.
View all literature mentionsMus musculus with name B6.Cg-Tg(SOD1*G93A)1Gur/J from IMSR.
View all literature mentionsCell line Lex-2 is a Embryonic stem cell with a species of origin Mus musculus (Mouse)
View all literature mentionsThis unknown targets α-subunit of the nicotinic acetylcholine receptor (AChR) of neuromuscular junction
View all literature mentionsThe SciCrunch Infrastructure was developed as a cooperative data platform to be used by diverse communities in making data more FAIR.
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