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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >Uncoupling of growth plate maturation and bone formation in mice lacking both Schnurri-2 and Schnurri-3
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Uncoupling of growth plate maturation and bone formation in mice lacking both Schnurri-2 and Schnurri-3

机译：缺乏Schnurri-2和Schnurri-3的小鼠生长板成熟和骨形成的解偶联

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摘要

Formation and remodeling of the skeleton relies on precise temporal and spatial regulation of genes expressed in cartilage and bone cells. Debilitating diseases of the skeletal system occur when mutations arise that disrupt these intricate genetic regulatory programs. Here, we report that mice bearing parallel null mutations in the adapter proteins Schnurri2 (Shn2) and Schnurri3 (Shn3) exhibit defects in patterning of the axial skeleton during embryo-genesis. Postnatally, these compound mutant mice develop a unique osteochondrodysplasia. The deletion of Shn2 and Shn3 impairs growth plate maturation during endochondral ossification but simultaneously results in massively elevated trabecular bone formation. Hence, growth plate maturation and bone formation can be uncoupled under certain circumstances. These unexpected findings demonstrate that both unique and redundant functions reside in the Schnurri protein family that are required for proper skeletal patterning and remodeling.
机译：骨骼的形成和重塑依赖于软骨和骨细胞中表达的基因的精确时空调节。当发生突变而破坏这些复杂的基因调控程序时，就会发生骨骼系统的衰弱性疾病。在这里，我们报告的小鼠在适配器蛋白Schnurri2（Shn2）和Schnurri3（Shn3）中带有平行无效突变的小鼠在胚胎生成过程中在轴向骨架的图案中显示出缺陷。出生后，这些复合突变小鼠发展出独特的骨软骨发育不良。 Shn2和Shn3的缺失会损害软骨内骨化过程中生长板的成熟，但同时会导致小梁骨的形成大量增加。因此，在某些情况下，生长板的成熟和骨骼的形成可能是分离的。这些出乎意料的发现表明，Schnurri蛋白家族既有独特的功能，也有多余的功能，这是适当的骨骼构图和重塑所必需的。

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  • 作者

    Dallas C. Jones; Michelle N. Schweitzer; Marc Wein; Kirsten Sigrist; Tsuyoshi Takagi; Shunsuke Ishii; Laurie H. Glimcher;

  • 作者单位

    Department of Immunology and Infectious Disease, Harvard School of Public Health, Boston, MA 02115 Department of Medicine, Harvard Medical School, Boston, MA 02115;

    rnDepartment of Immunology and Infectious Disease, Harvard School of Public Health, Boston, MA 02115;

    rnDepartment of Immunology and Infectious Disease, Harvard School of Public Health, Boston, MA 02115;

    rnDepartment of Immunology and Infectious Disease, Harvard School of Public Health, Boston, MA 02115;

    rnLaboratory of Molecular Genetics, RIKEN Tsukuba Institute, Tsukuba, Ibaraki 305-0074, Japan;

    rnLaboratory of Molecular Genetics, RIKEN Tsukuba Institute, Tsukuba, Ibaraki 305-0074, Japan;

    rnDepartment of Immunology and Infectious Disease, Harvard School of Public Health, Boston, MA 02115 Department of Medicine, Harvard Medical School, Boston, MA 02115 Ragon Institute of Massachusetts Institute of Technology, Massachusetts General Hospital, and Harvard, Boston, MA 02129;

  • 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
  • 关键词

    chondrodysplasia; osteoblast; skeletogenesis;

    机译：软骨发育不良成骨细胞骨骼生成;

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