Notochord-dependent expression of MFH1 and PAX1 cooperates to maintain the proliferation of sclerotome cells during the vertebral column development
Notochord-dependent expression of MFH1 and PAX1 cooperates to maintain the proliferation of sclerotome cells during the vertebral column development
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DOI：
10.1006/dbio.1999.9261
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发表时间：
1999-06-01
影响因子：
2.7
通讯作者：
Koseki, H
中科院分区：
文献类型：
--
作者：
Furumoto, T;Miura, N;Koseki, H
在中轴骨骼发育过程中，脊索对于诱导至关重要。用于随后分化形成椎体和椎间盘的软骨。这些功能主要由可扩散信号分子 Sonic humig 介导。含有配对盒的 Pax1 和间充质叉头-1 (Mfh1) 基因的产物在发育中的巩膜中表达，对于脊柱的正常发育至关重要。在这里，我们证明 Mfh1 与 Pax1 的表达类似，依赖于来自脊索的 Sonic Hedgehog 信号，并且 Mfh1 和 Pax1 协同作用以生成脊柱。 Mfh1/Pax1双突变体中，椎骨背内侧结构缺失，导致极度脊柱裂并伴有皮下脊髓脊膜膨出，椎体和椎间盘缺失。 Mfh1/Pax1双突变体的形态缺陷与巩膜细胞有丝分裂率的降低密切相关。因此，Mfh1 和 Pax1 基因产物共同介导 Sonic Hedgehog 依赖性的骨节细胞增殖。 (C) 1999 年学术出版社。
During axial skeleton development, the notochord is essential for the induction of. the sclerotome and for the subsequent differentiation of cartilage forming the vertebral bodies and intervertebral discs. These functions are mainly mediated by the diffusible signaling molecule Sonic hedgehog. The products of the paired-box-containing Pax1 and the mesenchyme forkhead-1 (Mfh1) genes are expressed in the developing sclerotome and are essential for the normal development of the vertebral column. Here, we demonstrate that Mfh1 like Pax1 expression is dependent on Sonic hedgehog signals from the notochord, and Mfh1 and Pax1 act synergistically to generate the vertebral column. In Mfh1/Pax1 double mutants, dorsomedial structures of the vertebrae are missing, resulting in extreme spina bifida accompanied by subcutaneous myelomeningocoele, and the vertebral bodies and intervertebral discs are missing. The morphological defects in Mfh1/Pax1 double mutants strongly correlate with the reduction of the mitotic rate of sclerotome cells. Thus, both the Mfh1 and the Pax1 gene products cooperate to mediate Sonic hedgehog-dependent proliferation of sclerotome cells. (C) 1999 Academic Press.