Abo A
Onyx Pharmaceuticals, Richmond, California 94806, USA.
Cell Mol Life Sci. 1998 Oct;54(10):1145-53. doi: 10.1007/s000180050242.
Wiskott-Aldrich syndrome (WAS) is an X-linked immunodeficiency disorder associated with lymphocytes and platelet abnormalities. The gene that encodes the Wiskott-Aldrich protein (WASP) was recently isolated, and shown to be defective in WAS patients. WASP contains multiple domains that interact with various signalling proteins, including the guanine triphosphatase (GTPase) Cdc42Hs and SH3 domain-containing proteins. Biochemical and genetic evidence strongly suggests that WASP is an important protein in the regulation of cell morphology. Recent progress in the identification of molecular partners for WASP suggests a molecular mechanism for the cellular abnormalities of WAS.
威斯科特-奥尔德里奇综合征（WAS）是一种与淋巴细胞和血小板异常相关的X连锁免疫缺陷疾病。编码威斯科特-奥尔德里奇蛋白（WASP）的基因最近已被分离出来，并显示在WAS患者中存在缺陷。WASP包含多个与各种信号蛋白相互作用的结构域，包括鸟嘌呤三磷酸酶（GTPase）Cdc42Hs和含SH3结构域的蛋白。生化和遗传学证据有力地表明，WASP是细胞形态调节中的一种重要蛋白。在确定WASP分子伴侣方面的最新进展提示了WAS细胞异常的分子机制。
