Petrich Brian G, Fogelstrand Per, Partridge Anthony W, Yousefi Nima, Ablooglu Ararat J, Shattil Sanford J, Ginsberg Mark H
Department of Medicine, UCSD School of Medicine, La Jolla, California 92093-0726, USA.
J Clin Invest. 2007 Aug;117(8):2250-9. doi: 10.1172/JCI31024.
In vitro studies indicate that binding of talin to the beta(3) integrin cytoplasmic domain (tail) results in integrin alpha(IIb)beta(3) (GPIIb-IIIa) activation. Here we tested the importance of talin binding for integrin activation in vivo and its biological significance by generating mice harboring point mutations in the beta(3) tail. We introduced a beta(3)(Y747A) substitution that disrupts the binding of talin, filamin, and other cytoplasmic proteins and a beta(3)(L746A) substitution that selectively disrupts interactions only with talin. Platelets from animals homozygous for each mutation showed impaired agonist-induced fibrinogen binding and platelet aggregation, providing proof that inside-out signals that activate alpha(IIb)beta(3) require binding of talin to the beta(3) tail. beta(3)(L746A) mice were resistant to both pulmonary thromboembolism and to ferric chloride-induced thrombosis of the carotid artery. Pathological bleeding, measured by the presence of fecal blood and development of anemia, occurred in 53% of beta(3)(Y747A) and virtually all beta(3)-null animals examined. Remarkably, less than 5% of beta(3)(L746A) animals exhibited this form of bleeding. These results establish that alpha(IIb)beta(3) activation in vivo is dependent on the interaction of talin with the beta(3) integrin cytoplasmic domain. Furthermore, they suggest that modulation of beta(3) integrin-talin interactions may provide an attractive target for antithrombotics and result in a reduced risk of pathological bleeding.
体外研究表明,踝蛋白与β3整合素胞质结构域(尾部)的结合会导致整合素α(IIb)β3(糖蛋白IIb-IIIa)激活。在此,我们通过构建β3尾部存在点突变的小鼠,测试了踝蛋白结合对于体内整合素激活的重要性及其生物学意义。我们引入了β3(Y747A)替代突变,该突变破坏了踝蛋白、细丝蛋白和其他胞质蛋白的结合,以及β3(L746A)替代突变,该突变仅选择性地破坏与踝蛋白的相互作用。每种突变纯合子动物的血小板显示出激动剂诱导的纤维蛋白原结合和血小板聚集受损,这证明激活α(IIb)β3的外向内信号需要踝蛋白与β3尾部结合。β3(L746A)小鼠对肺血栓栓塞和氯化铁诱导的颈动脉血栓形成均具有抗性。通过粪便潜血和贫血发展来衡量的病理性出血,在53%的β3(Y747A)小鼠以及几乎所有检测的β3基因缺失动物中出现。值得注意的是,不到5%的β3(L746A)动物表现出这种出血形式。这些结果表明,体内α(IIb)β3激活依赖于踝蛋白与β3整合素胞质结构域的相互作用。此外,它们表明调节β3整合素-踝蛋白相互作用可能为抗血栓药物提供一个有吸引力的靶点,并降低病理性出血的风险。