Mouse Ror2 receptor tyrosine kinase is required for the heart development and limb formation
Mouse Ror2 receptor tyrosine kinase is required for the heart development and limb formation
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DOI：
10.1046/j.1365-2443.2000.00300.x
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发表时间：
2000-01-01
期刊：
影响因子：
2.1
通讯作者：
Minami, Y
中科院分区：
文献类型：
--
作者：
Takeuchi, S;Takeda, K;Minami, Y
背景资料：小鼠受体酪氨酸激酶（RTK）mRor 2属于Ror家族，由至少两个结构相关的成员组成，主要在小鼠发育期间的心脏和神经系统中表达。为了阐明mRor 2的功能，我们产生了突变的mRor 2 locus.Results：mRor 2纯合突变的小鼠出生后立即死亡，表现出侏儒症，严重的发绀，短四肢和尾巴。整体原位杂交分析表明，mRor 2表达在鳃弓，心脏和肢体/尾芽，除了发育中的神经系统。这些突变体有心脏间隔缺损，主要是室间隔缺损。此外，对骨骼系统的检查显示，突变体的四肢、椎骨和面部结构较短，在其远端部分有一个特殊的缺陷，在其远端四肢几乎没有观察到钙化。组织学检查显示异常的软骨cytos.Conclusions：我们的研究结果表明，mRor 2起着至关重要的作用，在发展中的心脏和四肢/尾巴的形成，特别是心脏隔膜的形成和骨化的四肢和尾巴的远端部分。
Backgrounds: A mouse receptor tyrosine kinase (RTK), mRor2, which belongs to the Ror-family of RTKs consisting of at least two structurally related members, is primarily expressed in the heart and nervous system during mouse development. To elucidate the function of mRor2, we generated mice with a mutated mRor2 locus.Results: Mice with a homozygous mutation in mRor2 died just after birth, exhibiting dwarfism, severe cyanosis, and short limbs and tails. Whole-mount in situ hybridization analysis showed that mRor2 was expressed in the branchial arches, heart and limb/tailbuds, in addition to the developing nervous system. The mutants had cardiac septal defects, mainly a ventricular septal defect. In addition, an examination of the skeletal systems revealed that the mutants had shorter limbs, vertebrae and facial structure, with a particular defect in their distal portions, and that almost no calcification was observed in their distal limbs. Histological examination showed abnormalities in the chondrocytes.Conclusions: Our findings suggest that mRor2 plays essential roles in the development of the heart and in limb/tail formation, in particular cardiac septal formation and ossification of distal portions of limbs and tails.