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Kindlin-3 in the immune system

机译:免疫系统中的Kindlin-3

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摘要

Kindlin-3 is a member of the kindlin family of focal adhesion proteins which bind to integrin beta-chain cytoplasmic domains to regulate integrin function. In contrast to kindlin-1 and kindlin-2 proteins, kindlin-3 is expressed mainly in the hematopoietic system. Mutations in kindlin-3 result in the rare genetic disorder, leukocyte adhesion deficiency type III (LAD-III), which is characterized by bleeding and recurrent infections due to deficient beta1, beta2 and beta3 integrin activation in platelets and leukocytes. Here, we review the role of kindlin-3 in integrin activation and in different immune cell functions.
机译:Kindlin-3是kindlin家族的黏着斑蛋白的成员,该蛋白与整联蛋白β链胞质结构域结合以调节整联蛋白功能。与kindlin-1和kindlin-2蛋白相反,kindlin-3主要在造血系统中表达。 kindlin-3中的突变导致罕见的遗传疾病,白细胞粘附缺乏症III型(LAD-III),其特征是由于血小板和白细胞中β1,β2和β3整合素的激活不足而导致出血和反复感染。在这里,我们回顾了kindlin-3在整联蛋白激活和不同免疫细胞功能中的作用。

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