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首页> 外文期刊>Cell and Tissue Research >Trps1 is necessary for normal temporomandibular joint development
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Trps1 is necessary for normal temporomandibular joint development

机译：Trps1是正常的颞下颌关节发育所必需的

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摘要

Mutation of the human TRPS1 gene leads to trichorhinophalangeal syndrome (TRPS), which is characterized by an abnormal development of various organs including the craniofacial skeleton. Trps1 has recently been shown to be expressed in the jaw joints of zebrafish; however, whether Trps1 is expressed in the mammalian temporomandibular joint (TMJ), or whether it is necessary for TMJ development is unknown. We have analyzed (1) the expression pattern of Trps1 during TMJ development in mice and (2) TMJ development in Trps1 knockout animals. Trps1 is expressed in the maxillo-mandibular junction at embryonic day (E) 11.5. At E15.5, expression is restricted to the developing condylar cartilage and to the surrounding joint disc progenitor cells. In Trps1 knockout mice, the glenoid fossa of the temporal bone forms relatively normally but the condylar process is extremely small and the joint disc and cavities do not develop. The initiation of condyle formation is slightly delayed in the mutants at E14.5; however, at E18.5, the flattened chondrocyte layer is narrowed and most of the condylar chondrocytes exhibit precocious chondrocyte maturation. Expression of Runx2 and its target genes is expanded toward the condylar apex in the mutants. These observations underscore the indispensable role played by Trps1 in normal TMJ development in supporting the differentiation of disc and synoviocyte progenitor cells and in coordinating condylar chondrocyte differentiation.
机译：人TRPS1基因的突变会导致毛发三角型综合征（TRPS），其特征是包括颅面骨骼在内的各种器官发育异常。最近发现Trps1在斑马鱼的颚关节中表达。然而，是否在哺乳动物颞下颌关节（TMJ）中表达Trps1，或者是否需要发展TMJ是未知的。我们已经分析了（1）小鼠TMJ发育期间Trps1的表达模式，以及（2）Trps1敲除动物中TMJ的表达。 Trps1在胚胎天（E）11.5在上颌下颌交界处表达。在E15.5，表达仅限于发育中的con突软骨和周围的关节盘祖细胞。在Trps1基因敲除小鼠中，颞骨的盂状窝形成相对正常，但是con突过程非常小，并且关节盘和腔不发育。在E14.5的突变体中，con的形成开始略有延迟。但是，在E18.5时，扁平软骨细胞层变窄，大多数the突软骨细胞表现出早熟的软骨细胞成熟。 Runx2及其靶基因的表达在突变体中向toward突顶点扩展。这些发现强调了Trps1在正常TMJ发育中在支持椎间盘和滑膜祖细胞的分化以及协调con突软骨细胞分化中起着不可或缺的作用。

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