Molecular biology research concernig mandible face growth of Hemifacial Microsomia

内皮素-1 （ET-1）是一种有效的血管收缩剂，首次从培养的猪内皮细胞中纯化得到。最近，ET-1的基因靶向揭示了其在颅面结构（包括骨和软骨）形成中的作用。将ET-1基因缺失小鼠的纯合子（ET-1 (-l-)）与杂合子和野生型（ET-1(+/+, +/-)）的表型进行比较和检查后，发现1例下颌骨窦毛均呈全样本下颌骨中端结合不良，致上颌，且纯合子中耳廓发育不全，与上颌窦毛相似。骨软骨标本：下颌突关节等效部分类似颧弓投影完全缺失，外踝、鼓室环完全缺失，与中轴梅克尔软骨相似。血管运行：ET-1 （-l-）中存在异常分支舌动脉唇下过量分支，多为面动脉复数，形态与上颌上动脉相似。组织图像：在ET-1 （-l-）中未被接纳，但细胞间底物的增加被ET-1头侧咽弓角质层正下方的间充质细胞（+l*）接纳为活胎10.5 d。基因外观：虽然在ET-1 （-l-）中PAX3显性且PAX3在上颌突和下颌骨突均显性，但在上颌突和下颌骨突中PAX3显性和下颌骨突中比较HANDs。ET-1 （-l-）的异常集中在下颌突的结构起源和下颌的原始形式，被认为使ET-1从两个或多个上颌进入下颌的外部病理形态，并且在ET-1 （-l-）下颌突中基因的表现模式类似于上颌骨突，并通过作用于间质细胞生发层和调节dHAND， eHAND的外观，Pax3与神经嵴细胞的迁移有关。少

Endothelin-1 (ET-1) isa potent vasoconstrictor that was first purified from cultured porcine endothelial cells. Recently, gene targeting of ET-1 reveals its role in the formation of craniofacial structures including bones and cartilage.When the phenotype of ET-1 gene deletion mouse's homozygote (ET-1 (-l-)) and heterozygote and wild type (ET-1 (+/+, +/-))was compared and had been examined, the sinus hair of a lower jaw all opatry which presented the faulty union of the median of all sample lower jaw, making to the maxilla, and hypoplasia of the pinna in the homozygote and was similar to the maxillary sinus hair in addition was admitted.Bone and cartilage specimen : the mandibular process articularise quivalent part were similarto the arcus zygomaticus projection on complete deletion, the malleus, and the anulus tympanicus complete Loss, like in the axis Meckel cartilage.Running of blood vessel : In ET-1 (-l-), an abnormal offshoot lingua arteria labialis inferior excessive branch exist … More ed from the arteria facialis in the plural and the form was similar to the arteria maxillaris of the upper jaw.Tissue image : It was not admitted in ET-1 (-l-) though the increase of the substrate between cells was admitted for the viviparity 10.5 days by the mesenchymal cell right under the head side pharyngeal arch cuticle of ET-1 (+l*).Appearance of gene : HANDs was compared in the PAX3 domination and the mandibular process in the maxillar process though it was dominant and PAX3 was dominant in both maxillar process and mandibular processes in ET-1 (-l-).Abnormality of ET-1 (-l-)concentrated on the structure of the mandibular process origin and originality in the form of the lower jaw was thought to give ET-1 from the admission of two or more upper jaw externals pathomorphisms of the lower jaw and the pattern of manifestation of the gene in the ET-1 (-l-) mandibular process similar the processus maxillaris and well by acting on a mesenchymal cell the germinal layer and adjusting the appearance of dHAND, eHAND, and Pax3 to the migration from neural crest cell. Less