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WASP 家族蛋白:分子机制及其在人类疾病中的意义。

WASP family proteins: Molecular mechanisms and implications in human disease.

机构信息

Roy J. Carver Department of Biochemistry, Biophysics & Molecular Biology, Iowa State University, 2437 Pammel Drive, Ames, IA 50011, USA.

Roy J. Carver Department of Biochemistry, Biophysics & Molecular Biology, Iowa State University, 2437 Pammel Drive, Ames, IA 50011, USA.

出版信息

Eur J Cell Biol. 2022 Jun-Aug;101(3):151244. doi: 10.1016/j.ejcb.2022.151244. Epub 2022 Jun 1.

Abstract

Proteins of the Wiskott-Aldrich syndrome protein (WASP) family play a central role in regulating actin cytoskeletal dynamics in a wide range of cellular processes. Genetic mutations or misregulation of these proteins are tightly associated with many diseases. The WASP-family proteins act by transmitting various upstream signals to their conserved WH2-Central-Acidic (WCA) peptide sequence at the C-terminus, which in turn binds to the Arp2/3 complex to stimulate the formation of branched actin networks at membranes. Despite this common feature, the regulatory mechanisms and cellular functions of distinct WASP-family proteins are very different. Here, we summarize and clarify our current understanding of WASP-family proteins and how disruption of their functions is related to human disease.

摘要

Wiskott-Aldrich 综合征蛋白(WASP)家族的蛋白在调节广泛的细胞过程中的肌动蛋白细胞骨架动力学方面起着核心作用。这些蛋白的遗传突变或失调与许多疾病密切相关。WASP 家族蛋白通过将各种上游信号传递到其 C 末端保守的 WH2-中央-酸性(WCA)肽序列来发挥作用,该序列反过来与 Arp2/3 复合物结合,以刺激分支状肌动蛋白网络在膜上的形成。尽管具有这一共性,但不同 WASP 家族蛋白的调节机制和细胞功能却大不相同。在这里,我们总结并阐明了我们目前对 WASP 家族蛋白的理解,以及它们功能的破坏如何与人类疾病相关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/34d0/9357188/e51dde3921e5/nihms-1816468-f0001.jpg

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