无翅型小鼠乳房肿瘤病毒整合位点家族基因与唇腭裂

Wingless-type mice mammary tumor virus integration site family and cleft lip and palate

无翅型小鼠乳房肿瘤病毒整合位点家族（WNT）糖蛋白是一组调控细胞增殖、分化和极性以及细胞移动的信号分子，其多种家族成员与唇腭裂发生密切相关。WNT-3基因位于17q21，其蛋白质主要通过经典WNT／β＋连环蛋白通路调节唇和腭的发生。WNT-3A基因位于1q24，主要表达于胎盘、气管、肺和结缔组织，其蛋白质通过调控神经脊细胞和腭间质细胞，影响颅面的发生进程及腭融合。WNT5A基因位于3p21～14，其蛋白质通过Ror2介导在非经典信号转导通路中调节腭发生。WNT-9B基因位于17q21，主要表达于上颌突、中鼻突和侧鼻突的外胚层，敲除该基因会出现唇裂和腭裂。WNT-11基因位于11q13．5，其蛋白质表达于腭中线缘上皮细胞，通过促进程序性细胞死亡参与腭板融合，与FGFR1b之间相互拮抗调节腭发生。研究删瑾因，可为进一步揭示唇腭裂病因及其机制提供思路。

Wingless-type mice mammary tumour virus integration site family glycoprotein is a set of signal molecules regulating cell proliferation, differentiation, cell polarity and cell mobility. Most members of the family is involved in lip and palatal development, and closely related to cleft lip and palate. WNT-3 gene is located in chromosome 17q21, the protein mainly act through the canonical WNT/β-catenin pathway to regulate the development of the lip and palate. WNT- 3A gene is located in chromoosome lq24, and is mainly expressed in the placenta, trachea, lung and connective tissue, it＇s protein affect the palatal fusion process and craniofacial development by regulating palatal mesenchymal cells and neural crest cells. WNToSA gene is located in chromosome 3p21-14, it＇s protein regulate the development of the palate through the Ror2-mediated non-canonical signal pathway. WNT-9B gene is located in chromosome 17@ 1, and is mainly expressed in the maxillary process, medial nasal process, and the ectoderm of the lateral nasal process, knock out of the gene will lead to cleft lip and cleft palate. WNT-11 gene is located in chromosome 1 lq13.5, it＇s protein is expressed the medial edge epithelium, affecting the palatal fusion by promoting programmed cell death, the cross-talk of WNT-11/FGFRlbregulates the development of the palate. Studying the WNT family can provide further ideas to reveal the cause and mechanism of cleft lip and palate.