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J Dent Res：循环中CNP可以改善软骨发育不全中的颅面发育不良

2017-06-26 lishiting MedSci原创

CNP颅颌面软骨发育不全
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软骨发育不全是人侏儒症中最常见的遗传形式，它具有面中部发育不良的特点，导致咬合异常和枕骨大孔狭窄，进一步引起严重的神经系统并发症和脑积水。目前，手术是治疗软骨发育不全患者颌骨畸形、神经系统并发症和脑积水的唯一方法。作者之前的研究显示，C型利钠肽(CNP)是长骨和椎骨内软骨生长强有力的刺激物，同时也是颌面区骨生长强有力的刺激物，它对面中部的骨发生起到至关重要的作用。

软骨发育不全是人侏儒症中最常见的遗传形式，它具有面中部发育不良的特点，导致咬合异常和枕骨大孔狭窄，进一步引起严重的神经系统并发症和脑积水。目前，手术是治疗软骨发育不全患者颌骨畸形、神经系统并发症和脑积水的唯一方法。作者之前的研究显示，C型利钠肽(CNP)是长骨和椎骨内软骨生长强有力的刺激物，同时也是颌面区骨生长强有力的刺激物，它对面中部的骨发生起到至关重要的作用。

在这项研究中，作者通过在软骨内特异性激活成纤维生长因子受体3(FGFR3)构建软骨发育不全的小鼠模型(Fgfr3ach小鼠)，并分析和探讨此种突变造成颌骨畸形的机制。另外，研究者也检测了CNP对此种突变小鼠上颌面区域的作用。

结果显示，由于颅面软骨组织的软骨内成骨受损以及颅颌面骨发生中的一个重要生长中心-蝶枕软骨结合的过早关闭，导致Fgfr3ach小鼠的面中部发育不良，特别是在矢状面。另外，研究者使用由人血清淀粉样蛋白P成分启动子驱动CNP在肝中表达的转基因小鼠与Fgfr3ach小鼠杂交获得Fgfr3ach/SAP-Nppc-Tg小鼠，促使循环中CNP表达上调。随后观察发现，在Fgfr3ach小鼠的后代中，由于软骨联合厚度的恢复以及颅面软骨内软骨细胞的显著增殖，使矢状面的面中部发育不良得到明显改善。除此之外，在Fgfr3ach小鼠中发现的枕骨大孔狭窄由于枕内前结合的软骨生长增强，在Fgfr3ach/SAP-Nppc-Tg小鼠中得到明显改善。

结论：这些结果清晰的阐明，CNP对于软骨发育不全中的面中部发育不良和枕骨大孔狭窄具有很大的治疗潜力。

原始出处：

Yamanaka S， Nakao K， et al. Circulatory CNP Rescues Craniofacial Hypoplasia in Achondroplasia. J Dent Res. 2017 Jun 1：22034517716437. doi： 10.1177/0022034517716437.

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    2018-01-13 minhuang75_72521594

    #CNP#

    0

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    2017-06-28 neurowu

    #发育#

    0

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    2017-06-28 villahu

    #发育不良#

    0

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    2017-06-27 184****9840

    学习了谢谢分享

    0

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    2017-06-26 清风拂面

    谢谢分享，学习了

    0