Редактирование: FGFBP1 (раздел)

==Publications==

{{medline-entry
|title=Muscle Fibers Secrete [[FGFBP1]] to Slow Degeneration of Neuromuscular Synapses during Aging and Progression of ALS.
|pubmed-url=https://pubmed.ncbi.nlm.nih.gov/28053031
|abstract=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 [[SOD1]]  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 [[SOD1]]  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. The neuromuscular junction (NMJ) is critical for all voluntary movement. Its malformation during development and degeneration in adulthood impairs motor function. Therefore, it is important to identify factors that function to maintain the structural integrity of NMJs. We show that muscle fibers secrete and concentrate the fibroblast growth factor binding protein 1 ([[FGFBP1]]) at NMJs. However, [[FGFBP1]] expression decreases in skeletal muscles during aging and before NMJ degeneration in [[SOD1]]  mice, a mouse model for amyotrophic lateral sclerosis. We show that transforming growth factor-β1 is responsible for the decreased levels of [[FGFBP1]]. Importantly, we demonstrate critical roles for [[FGFBP1]] at NMJs in developing, aging and [[SOD1]]  mice.
|mesh-terms=* Aging
* Amyotrophic Lateral Sclerosis
* Animals
* Axons
* Carrier Proteins
* Intercellular Signaling Peptides and Proteins
* Intracellular Signaling Peptides and Proteins
* Mice
* Mice, Knockout
* Muscle Fibers, Skeletal
* Muscle, Skeletal
* Nerve Degeneration
* Nerve Regeneration
* Neuromuscular Junction
* Superoxide Dismutase-1
* Synapses
* Transforming Growth Factor beta1
|keywords=* Lou Gehrig's disease
* axonal regeneration
* cytokine
* geriatric
* synaptic elimination
* target-derived
|full-text-url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5214636
}}