Essential pro-Bmp roles of crossveinless 2 in mouse organogenesis
Essential pro-Bmp roles of crossveinless 2 in mouse organogenesis
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DOI：
10.1242/dev.02647
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发表时间：
2006-11-15
期刊：
影响因子：
4.6
通讯作者：
Sasai, Yoshiki
中科院分区：
文献类型：
--
作者：
Ikeya, Makoto;Kawada, Masako;Sasai, Yoshiki
我们在这里报告的BMP结合蛋白crossveinless 2（Cv 2; Bmper）在小鼠器官发生的重要作用。在无效Cv 2突变小鼠中，原肠胚形成正常发生，但在表达Cv 2的组织如骨骼中发现了许多缺陷。在培养的Cv 2（-/-）成纤维细胞中，通过Bmp 4处理的软骨分化减少。此外，Cv 2（-/-）小鼠的脊柱和眼睛中的缺陷通过缺失Bmp 4基因的一个拷贝而显著增强，表明Cv 2在这些器官的发育中的促BMP作用。此外，Cv 2（-/-）突变体在内部器官形成的BMP依赖性过程中表现出实质性缺陷，例如肾脏中的肾单位生成。这种肾发育不全通过额外缺失编码结构上与Cv 2相关的pro-Bmp蛋白的Kcp（Cv 2）而协同增强。这项研究表明，在哺乳动物器官发生的多个方面的局部限制性信号增强的Cv 2的基本pro-Bmp功能。
We here report essential roles of the Bmp-binding protein crossveinless 2 (Cv2; Bmper) in mouse organogenesis. In the null Cv2 mutant mouse, gastrulation occurs normally, but a number of defects are found in Cv2-expressing tissues such as the skeleton. Cartilage differentiation by Bmp4 treatment is reduced in cultured Cv2(-/-) fibroblasts. Moreover, the defects in the vertebral column and eyes of the Cv2(-/-) mouse are substantially enhanced by deleting one copy of the Bmp4 gene, suggesting a pro-Bmp role of Cv2 in the development of these organs. In addition, the Cv2(-/-) mutant exhibits substantial defects in Bmp-dependent processes of internal organ formation, such as nephron generation in the kidney. This kidney hypoplasia is synergistically enhanced by the additional deletion of Kcp (Crim2) which encodes a pro-Bmp protein structurally related to Cv2. This study demonstrates essential pro-Bmp functions of Cv2 for locally restricted signal enhancement in multiple aspects of mammalian organogenesis.