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成纤维细胞生长因子信号在颅面发育及发育障碍中的作用

FGF signalling in craniofacial development and developmental disorders.

作者信息

Nie X, Luukko K, Kettunen P

机构信息

Section of Anatomy and Cell Biology, Department of Biomedicine, University of Bergen, Bergen, Norway.

出版信息

Oral Dis. 2006 Mar;12(2):102-11. doi: 10.1111/j.1601-0825.2005.01176.x.

Abstract

The Fgf signalling pathway is highly conserved in evolution and plays crucial roles in development. In the craniofacial region, it is involved in almost all structure development from early patterning to growth regulation. In craniofacial skeletogenesis, the Fgf signal pathway plays important roles in suture and synchondrosis regulation. Mutations of FGF receptors relate to syndromatic and non-syndromatic craniosynostosis. The Fgf10/Fgfr2b signal loop is critical for palatogenesis and submandibular gland formation. Perturbation of the Fgf signal is a possible mechanism of palatal cleft. Fgf10 haploinsufficiency has been identified as the cause of autosomal dominant aplasia of lacrimal and salivary glands. The Fgf signal is also a key regulator of tooth formation: in the absence of Fgfr2b tooth development is arrested at the bud stage. Fgfr4 has recently been identified as the key signal mediator in myogenesis. In this review, these aspects are discussed in detail with a focus on the most recent advances.

摘要

Fgf信号通路在进化过程中高度保守,在发育过程中发挥着关键作用。在颅面部区域,它几乎参与了从早期模式形成到生长调节的所有结构发育。在颅面骨骼发生过程中,Fgf信号通路在缝合和软骨联合调节中发挥重要作用。FGF受体的突变与综合征性和非综合征性颅缝早闭有关。Fgf10/Fgfr2b信号环对腭发育和下颌下腺形成至关重要。Fgf信号的扰动是腭裂的一种可能机制。Fgf10单倍体不足已被确定为常染色体显性泪腺和唾液腺发育不全的原因。Fgf信号也是牙齿形成的关键调节因子:在没有Fgfr2b的情况下,牙齿发育在芽阶段停止。Fgfr4最近被确定为肌生成中的关键信号介质。在本综述中,将详细讨论这些方面,并重点关注最新进展。

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