Hội chứng rối loạn truyền tin WAS

Mình k tìm thấy nội dung tiếng Việt, nếu bạn muốn đọc tiếng Anh thì tìm kiếm từ khóa Wiskott Aldrich syndrome (WAS)
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The importance of the relay proteins that serve as points of branching or intersection in signaling pathways is highlighted by the problems arising when these proteins are defective or missing. For instance, in an inherited disorder called WiskottAldrich syndrome (WAS), the absence of a single relay protein leads to such diverse effects as abnormal bleeding, eczema, and a predisposition to infections and leukemia. These symptoms are thought to arise primarily from the absence of the protein in cells of the immune system. By studying normal cells, scientists found that the WAS protein is located just beneath the cell surface. The protein interacts both with microfilaments of the cytoskeleton and with several different components of signaling pathways that relay information from the cell surface, including pathways regulating immune cell proliferation. This multifunctional relay protein is thus both a branch point and an important intersection point in a complex signal transduction network that controls immune cell behavior. When the WAS protein is absent, the cytoskeleton is not properly organized and signaling pathways are disrupted, leading to the WAS symptoms.


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