Comparison of hedgehog and patched-1 protein expression in the cranial sutures of craniosynostotic and wild-type rabbits.
Comparison of hedgehog and patched-1 protein expression in the cranial sutures of craniosynostotic and wild-type rabbits.
复制标题
颅缝早闭和野生型兔颅骨缝中刺猬蛋白和 patch-1 蛋白表达的比较。
DOI：
10.1097/00006534-200208000-00023
复制
发表时间：
2002
影响因子：
3.6
通讯作者：
Mooney,MarkP
中科院分区：
文献类型：
--
作者：
Nott,RhodaL;Stelnicki,EricJ;Mack,JudithA;Ben,Yixin;Mitchell,Ronal;Mooney,MarkP
颅缝早闭的特征是颅缝过早融合。在分子水平上，同源异型盒基因、转录因子和生长因子受体的突变与这种疾病的发病机制有关，但具体的致病途径尚未阐明。为了进一步研究颅缝早闭背后的分子生物学，在一个独特的先天性迟发性冠状颅缝早闭兔模型的缝周组织进行了检查，以确定是否存在刺猬家族的生长因子及其受体patched-1。沙漠刺猬，印度刺猬，音刺猬，补丁-1的表达进行了评估，在四个领域：缝，骨内膜，骨膜和骨细胞，使用免疫组织化学（n= 8）。将患病动物的蛋白质水平与野生型对照兔（n= 8）的蛋白质水平进行比较。总的来说，受影响的动物中的音速刺猬，印度刺猬和补丁-1蛋白水平更高。具体而言，在骨内膜和骨膜的骨界面以及骨性结合兔的骨细胞中沿着观察到染色增强的区域。有趣的是，在缝线中，观察到印度刺猬和音速刺猬水平增加，但未观察到patched-1。在两种兔类型中均存在最小的沙漠刺猬表达。hedgehog和patched-1蛋白在骨性结合兔中的总体存在增加可能是疾病的反应性变化或致病过程的一部分。虽然无法从数据中确定具体原因，但很明显，骨性结合兔颅缝的分子环境与野生型兔明显不同。（Plast。重建110：515，2002）。
: Craniosynostosis is characterized by premature fusion of the cranial sutures. At the molecular level, mutations in homeobox genes, transcription factors, and growth factor receptors have been implicated in the pathogenesis of this disorder, but the specific etiologic pathways have not yet been elucidated. To further study the molecular biology behind craniosynostosis, perisutural tissues in a unique rabbit model with congenital delayed-onset coronal craniosynostosis were examined for the presence of the hedgehog family of growth factors and their receptor, patched-1. Expression of desert hedgehog, Indian hedgehog, sonic hedgehog, and patched-1 was evaluated in four areas: suture, endosteum, periosteum, and osteocytes, using immuno-histochemistry (n= 8). Protein levels in affected animals were compared with protein levels in wild-type control rabbits (n= 8). Overall, sonic hedgehog, Indian hedgehog, and patched-1 protein levels were greater in affected animals. Specifically, areas of increased staining were seen along the bony interface of the endosteum and periosteum and in the osteocytes of the synostotic rabbits. Interestingly, in the suture, increased levels of Indian hedgehog and sonic hedgehog, but not patched-1, were seen. There was minimal expression of desert hedgehog in both rabbit types. The increased overall presence of hedgehog and patched-1 proteins in synostotic rabbits may be a reactive change to the disorder or part of the pathogenic process. Although the specific cause cannot be determined from the data, it is clear that the molecular milieu of the cranial sutures in synostotic rabbits is markedly different from that of wild-type rabbits.(Plast. Reconstr. Surg. 110: 515, 2002.)