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首页> 外文期刊>Journal of Cellular Physiology >Control of lung development by latent TGF-beta binding proteins.
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Control of lung development by latent TGF-beta binding proteins.

机译：控制肺发展潜在的及结合蛋白。

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The latent TGF-beta binding proteins (LTBP-1 -3, and -4) assist in the secretion and localization of latent TGF-beta molecules. Ltbp3(-/-) and Ltbp4S(-/-) mice have distinct phenotypes and only in the lungs does deficiency of either Ltbp-3 or Ltbp-4 cause developmental abnormalities. To determine if these two LTBPs have additional common functions, we generated mice deficient for both Ltbp-3 and Ltbp-4S. The only novel defect in Ltbp3(-/-);Ltbp4S(-/-) mice was an early lethality compared to mice with single mutations. In addition lung abnormalities were exacerbated and the terminal air sac septation defect was more severe in Ltbp3(-/-);Ltbp4S(-/-) mice than in Ltbp4S(-/-) mice. Decreased cellularity of Ltbp3(-/-);Ltbp4S(-/-) lungs was correlated with higher rate of apoptosis in newborn lungs of Ltbp3(-/-);Ltbp4S(-/-) animals compared to WT, Ltbp3(-/-), and Ltbp4S(-/-) mice. No differences in the maturation of the major lung cell types were discerned between the single and double mutant mice. However, the distribution of type 2 cells and myofibroblasts was abnormal, and myofibroblast segregation in some areas might be an indication of early fibrosis. We also observed differences in ECM composition between Ltbp3(-/-);Ltbp4S(-/-) and Ltbp4S(-/-) lungs after birth, reflected in decreased incorporation of fibrillin-1 and -2 in Ltbp3(-/-);Ltbp4S(-/-) matrix. The function of the lungs of Ltbp3(-/-);Ltbp4S(-/-) mice after the first week of life was potentially further compromised by macrophage infiltration, as proteases secreted from macrophages might exacerbate developmental emphysema. Together these data indicate that LTBP-3 and -4 perform partially overlapping functions only in the lungs.
机译：的鉴定及结合蛋白(LTBP-1 3,4)协助分泌和本地化潜在的鉴定及分子。Ltbp4S(- / -)小鼠有明显的表型只有在肺部也不足Ltbp-3或Ltbp-4造成发育异常。我们有额外的通用功能,生成的吗老鼠Ltbp-3和Ltbp-4S不足。只有小说在Ltbp3缺陷(- / -);Ltbp4S(- / -)小鼠是一个早期的杀伤力比老鼠单突变。加剧,终端空气囊分隔作用缺陷更严重老鼠。Ltbp3 (- / -); Ltbp4S(- / -)与肺新生儿肺的细胞凋亡率高Ltbp3 (- / -); Ltbp4S(- / -)动物WT相比,Ltbp3 (- / -), Ltbp4S(- / -)小鼠。成熟的大肺细胞类型单引号和双间被看见突变的老鼠。细胞和myofibroblasts异常,myofibroblast隔离在某些领域表明早期纤维化。ECM成分之间的差异出生后,反映在合并下降在Ltbp3 fibrillin-1和2 (- / -);Ltbp4S (- / -)矩阵。Ltbp3 (- / -); Ltbp4S(- / -)小鼠后第一周的生活可能进一步损害巨噬细胞浸润,如蛋白酶分泌从巨噬细胞可能会加剧发展肺气肿。LTBP-3和4执行部分重叠只在肺部功能。

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