Shox2-deficiency leads to dysplasia and ankylosis of the temporomandibular joint in mice
Shox2-deficiency leads to dysplasia and ankylosis of the temporomandibular joint in mice
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Shox2缺陷导致小鼠颞下颌关节发育不良和强直
DOI：
10.1016/j.mod.2008.04.003
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发表时间：
2008-08-01
影响因子：
2.6
通讯作者：
Chen, YiPing
中科院分区：
文献类型：
--
作者：
Gu, Shuping;Wei, Na;Chen, YiPing
颞下颌关节（TMJ）是一种独特的滑膜关节，其发育不同于其他滑膜关节的形成。只有少数基因的突变与颞下颌关节发育缺陷有关。在这项研究中，我们报道了同源盒基因Shox2在上颌交界处的颅神经嵴来源的间充质细胞中表达，随后在发育中的TMJ的髁突的祖细胞和未分化软骨细胞以及关节盂窝中表达。颅神经嵴源性细胞中Shox2的条件性失活导致TMJ发育异常，包括髁突和盂窝发育不良。关节盘形成，但与髁突和盂窝的纤维层融合，临床上称为TMJ强直。组织学检查显示突变的TMJ发育迟缓，并伴有细胞增殖率显著降低。原位杂交进一步证明了几个关键成骨基因的表达改变和成骨分化标志物的表达延迟。Shox2似乎调节成骨基因的表达，对TMJ的发育和功能至关重要。因此，Shox2条件突变体提供了一个独特的TMJ强直动物模型。2008爱思唯尔爱尔兰有限公司版权所有。
The temporomandibular joint (TMJ) is a unique synovial joint whose development differs from the formation of other synovial joints. Mutations have been associated with the developmental defects of the TMJ only in a few genes. in this study, we report the expression of the homeobox gene Shox2 in the cranial neural crest derived mesenchymal cells of the maxilla-mandibular junction and later in the progenitor cells and undifferentiated chondrocytes of the condyle as well as the glenoid fossa of the developing TMJ. A conditional inactivation of Shox2 in the cranial neural crest-derived cells causes developmental abnormalities in the TMJ, including dysplasia of the condyle and glenoid fossa. The articulating disc forms but fuses with the fibrous layers of the condyle and glenoid fossa, clinically known as TMJ ankylosis. Histological examination indicates a delay in development in the mutant TMJ, accompanied by a significantly reduced rate of cell proliferation. in situ hybridization further demonstrates an altered expression of several key osteogenic genes and a delayed expression of the osteogenic differentiation markers. Shox2 appears to regulate the expression of osteogenic genes and is essential for the development and function of the TMJ. The Shox2 conditional mutant thus provides a unique animal model of TMJ ankylosis. (C) 2008 Elsevier Ireland Ltd. All rights reserved.