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首页> 外文期刊>Molecular and Cellular Biology >Fatal Bilateral Chylothorax in Mice Lacking the Integrin α9β1
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Fatal Bilateral Chylothorax in Mice Lacking the Integrin α9β1

机译：缺少整联蛋白α9β1的小鼠的致命性双侧胸廓

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Members of the integrin family of adhesion receptors mediate both cell-cell and cell-matrix interactions and have been shown to play vital roles in embryonic development, wound healing, metastasis, and other biological processes. The integrin α9β1 is a receptor for the extracellular matrix proteins osteopontin and tenacsin C and the cell surface immunoglobulin vascular cell adhesion molecule-1. This receptor is widely expressed in smooth muscle, hepatocytes, and some epithelia. To examine the in vivo function of α9β1, we have generated mice lacking expression of the α9 subunit. Mice homozygous for a null mutation in the α9 subunit gene appear normal at birth but develop respiratory failure and die between 6 and 12 days of age. The respiratory failure is caused by an accumulation of large volumes of pleural fluid which is rich in triglyceride, cholesterol, and lymphocytes. α9 ?/? mice also develop edema and lymphocytic infiltration in the chest wall that appears to originate around lymphatics. α9 protein is transiently expressed in the developing thoracic duct at embryonic day 14, but expression is rapidly lost during later stages of development. Our results suggest that the α9 integrin is required for the normal development of the lymphatic system, including the thoracic duct, and that α9 deficiency could be one cause of congenital chylothorax.
机译：整合素家族粘附受体的成员介导细胞与细胞和细胞与基质的相互作用，并已显示在胚胎发育，伤口愈合，转移和其他生物学过程中起着至关重要的作用。整联蛋白α9β1是细胞外基质蛋白骨桥蛋白和腱糖蛋白C以及细胞表面免疫球蛋白血管细胞粘附分子-1的受体。该受体在平滑肌，肝细胞和一些上皮细胞中广泛表达。为了检查α9β1的体内功能，我们产生了缺乏α9亚基表达的小鼠。纯合α9亚基突变的小鼠在出生时看起来正常，但会出现呼吸衰竭并在6至12天龄时死亡。呼吸衰竭是由大量富含甘油三酸酯，胆固醇和淋巴细胞的胸膜积液引起的。 α 9 ？/？小鼠的胸壁也出现水肿和淋巴细胞浸润，似乎起源于淋巴管周围。在胚胎的第14天，α9蛋白在发育中的胸导管中短暂表达，但在发育的后期阶段表达迅速丧失。我们的结果表明，α9整联蛋白是淋巴系统（包括胸导管）正常发育所必需的，并且α9缺乏可能是先天性乳糜胸的原因之一。

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