A genetic model for a central (septum transversum) congenital diaphragmatic hernia in mice lacking Slit3.

Yuan W; Rao Y; Babiuk RP; Greer J; Wu JY; Ornitz DM

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A genetic model for a central (septum transversum) congenital diaphragmatic hernia in mice lacking Slit3.

机译：缺少Slit3的小鼠中枢（横隔）先天性diaphragm肌疝的遗传模型。

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摘要

Congenital diaphragmatic hernia (CDH) is a significant cause of pediatric mortality in humans with a heterogeneous and poorly understood etiology. Here we show that mice lacking Slit3 developed a central (septum transversum) CDH. Slit3 encodes a member of the Slit family of guidance molecules and is expressed predominantly in the mesothelium of the diaphragm during embryonic development. In Slit3 null mice, the central tendon region of the diaphragm fails to separate from liver tissue because of abnormalities in morphogenesis. The CDH progresses through continuous growth of the liver into the thoracic cavity. This study establishes the first genetic model for CDH and identifies a previously unsuspected role for Slit3 in regulating the development of the diaphragm.

机译：先天性diaphragm肌疝（CDH）是病因不明且知之甚少的小儿死亡率的重要原因。在这里，我们显示缺少Slit3的小鼠发展了中央（横隔）CDH。 Slit3编码Slit家族的指导分子的成员，并在胚胎发育过程中主要在隔膜的间皮中表达。在Slit3空小鼠中，由于形态发生异常，横the膜的中央腱区域无法与肝脏组织分离。 CDH通过肝脏持续生长进入胸腔而发展。这项研究建立了CDH的第一个遗传模型，并确定了Slit3在调节横diaphragm膜发育中以前没有想到的作用。

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来源
《Proceedings of the National Academy of Sciences of the United States of America》 |2003年第9期|P.5217-5222|共6页
作者
Yuan W; Rao Y; Babiuk RP; Greer J; Wu JY; Ornitz DM;
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作者单位

Departments of Molecular Biology and Pharmacology, Anatomy and Neurobiology, and Pediatrics, Washington University School of Medicine, St. Louis, MO 63110.;

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收录信息美国《科学引文索引》(SCI);美国《生物学医学文摘》(MEDLINE);美国《化学文摘》(CA);
原文格式 PDF
正文语种 eng
中图分类自然科学总论;
关键词
Laboratory mice; Models, Genetic; Diaphragm (Anatomy); Hernia, Diaphragmatic; 模型, 遗传学; 疝, 横膈;

机译：Laboratory mice;Models, Genetic;Diaphragm (Anatomy);Hernia, Diaphragmatic;模型, 遗传学;疝, 横膈;

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1. Conditional deletion of WT1 in the septum transversum mesenchyme causes congenital diaphragmatic hernia in mice [J] . Rita Carmona, Ana Ca?ete, Elena Cano, eLife journal . 2016,第september期

机译：间隔横隔间质中WT1的有条件缺失导致小鼠先天性diaphragm肌疝
2. Mouse lacking COUP-TFII as an animal model of Bochdalek-type congenital diaphragmatic hernia. [J] . You LR, Takamoto N, Yu CT, Proceedings of the National Academy of Sciences of the United States of America . 2005,第45期

机译：缺少COUP-TFII的小鼠是Bochdalek型先天性diaphragm肌疝的动物模型。
3. Teratogen-induced, dietary and genetic models of congenital diaphragmatic hernia share a common mechanism of pathogenesis. [J] . Clugston RD, Klattig J, Englert C, American Journal of Pathology: Official Publication of the American Association of Pathologists . 2006,第5期

机译：致畸剂，先天性ogen肌疝的饮食和遗传模型具有共同的发病机理。
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5. Investigating the mechanisms underlying congenital diaphragmatic hernia and lung maturation. [D] . Branchfield, Kelsey. 2015

机译：研究先天性diaphragm肌疝和肺成熟的潜在机制。
6. A genetic model for a central (septum transversum) congenital diaphragmatic hernia in mice lacking Slit3 [O] . Wenlin Yuan, Yi Rao, Randal P. Babiuk, 2003

机译：缺少Slit3的小鼠中枢（横隔）先天性diaphragm肌疝的遗传模型
7. A genetic model for a central (septum transversum) congenital diaphragmatic hernia in mice lacking Slit3 [O] . Yuan, Wenlin, Rao, Yi, Babiuk, Randal P., 2003

机译：缺少Slit3的小鼠中枢（横隔）先天性diaphragm肌疝的遗传模型

A genetic model for a central (septum transversum) congenital diaphragmatic hernia in mice lacking Slit3.

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