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阻断FGF23信号改善了X结合低酸血症小鼠的生长板

Rocío Fuente^1,2,3, Eva-Maria Pastor-Arroyo¹, Nicole Gehring¹

¹Institute of Physiology, University of Zurich, and National Center of Competence in Research NNCR Kidney, Zurich, CH, Switzerland.

The Journal of endocrinology

|July 13, 2023

概括

纤维细胞生长因子23 (FGF23) 的新型C终端碎片在X链接低度病 (XLH) 的小鼠模型中改善了生长和骨结构. 这些FGF23为XLH患者提供了潜在的新治疗途径.

科学领域:

内分泌学在内分泌学.
遗传学遗传学是一个
整形外科整形外科整形外科

背景情况:

链接到X的低酸性血症 (XLH) 是一种遗传性疾病,由于PHEX基因突变,导致酸盐浪费和骨矿化受损.
纤维细胞生长因子23 (FGF23) 水平升高是XLH的标志,导致生长障碍.
目前针对FGF23的治疗方法虽然有效,但成本高昂,并非普遍成功.

研究的目的:

在XLH小鼠模型中研究C端FGF23 (cFGF23) 片段的治疗潜力.
评估改性cFGF23对年轻XLH小鼠生长和骨参数的影响.

主要方法:

治疗年轻的Phex C733RMhda (XLH模型) 小鼠,用短cFGF23片段和两个修饰cFGF23进行7天的治疗.
分析血液参数,骨长,生长板结构和冠状细胞动态.

主要成果:

没有观察到血液参数的显著变化.
在接受治疗的小鼠中,观察到骨长度和生长板结构的显著改善.
观察到加速的生长率和部分恢复的冠状细胞的增殖,死亡,过度缩小,和软骨组成.

结论:

短cFGF23片段,特别是改性,在改善XLH的生长和骨缺陷方面表现有前途.
这些发现突显了FGF23信号在生长板代谢中的作用,即使存在持续的低酸血症.
cFGF23代表了XLH的潜在替代或辅助疗法,需要进一步调查.

关键词:

联邦政府财政基金23 XLH XLH 在线观看骨质密度骨质密度骨质密度增长的增长增长的增长增长的增长.增长板的增长板是什么

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