The role of the fibroblast growth factor receptor in the growth of the condylar cartilage and function maintenance

Muenke综合征以各种颅面畸形为特征，是由成纤维细胞生长因子受体3的常染色体显性显性激活突变引起的。在这里，我们通过携带相应突变的小鼠来确定该突变是否影响颞下颌关节的发育和生长。原位杂交显示，FGFR3在表达印度Hedgehog受体和转录靶标Patch 1的成熟期软骨细胞和成熟期软骨细胞中正常表达。在Fgfr3突变体中，髁状突生长板紊乱，表现为IHH表达水平降低，H4C阳性增殖软骨。我们还观察到TMJ关节隆起关节窝发育受阻。我们的数据表明，激活的FgfR3突变可能通过减少Hedgehog信号和软骨内成骨而干扰正常的TMJ发育过程。

Muenke syndrome is characterized by various craniofacial deformities and is caused by an autosomal dominant activating mutation in fibroblast growth factor receptor 3. Here we determined whether the mutation affects temporomandibular joint development and growth using mice carrying a corresponding mutation. In situ hybridization showed that Fgfr3 is normally expressed in condylar chondroprogenitors and maturing chondrocytes that expressed also the Indian hedgehog receptor and transcriptional target Patched 1.In Fgfr3mutants, the condylar growth plates were disorganized and displayed reduced levels of Ihh expression, H4C-positive proliferating chonWe also observed disrupted development of TMJ articular eminence glenoid fossa.droprogenitors, and collagens type II- and type X-expressing chondrocytes. Our data indicate that the activating FgfR3mutation disturbs normal TMJ developmental processes likely by reducing hedgehog signaling and endochondral ossification.