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[成纤维细胞生长因子受体与软骨发育不全]

[Fibroblast growth factor receptor and achondroplasia].

作者信息

Tanaka Hiroyuki

机构信息

Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Department of Pediatrics.

出版信息

Clin Calcium. 2006 Nov;16(11):1888-93.

Abstract

Fibroblast growth factor receptor 3 (FGFR3) has been establishing its position in growth plate cartilage after the identification as a responsible gene for achondroplasia. The major pathway of the pathogenesis in achondroplasia is the suppression of parathyroid hormone-related protein (PTHrP)-parathyroid hormone receptor (PTHR) system, which is mainly mediated by extracellular signal regulated kinase (ERK) activation induced by constitutive active FGFR3. However, intracellular signaling system in FGFR3 is complex and the molecular pathogenesis of achondroplasia and related disorders has not been fully clarified. In this review, I summarized recent consensus in the pathogenesis of FGFR3 related chondrodysplasia.

摘要

成纤维细胞生长因子受体3(FGFR3)在被鉴定为软骨发育不全的致病基因后,已在生长板软骨中确立了其地位。软骨发育不全发病机制的主要途径是甲状旁腺激素相关蛋白(PTHrP)-甲状旁腺激素受体(PTHR)系统的抑制,这主要由组成型活性FGFR3诱导的细胞外信号调节激酶(ERK)激活介导。然而,FGFR3的细胞内信号系统很复杂,软骨发育不全及相关疾病的分子发病机制尚未完全阐明。在本综述中,我总结了FGFR3相关软骨发育异常发病机制的最新共识。

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