Disease mechanism - Unravelling Wiskott-Aldrich syndrome

Disease mechanism - Unravelling Wiskott-Aldrich syndrome
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DOI:
10.1016/s0960-9822(09)00447-3
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发表时间:
1996-06-01
期刊:
影响因子:
9.2
通讯作者:
Rosen, FS
Rosen, FS
中科院分区:
生物学1区
文献类型:
--
作者:
Kirchhausen, T;Rosen, FS

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负责Wiskott-Aldrich综合征(一种影响血小板和淋巴细胞的疾病)的基因已被克隆,其蛋白质产物(WASp)被发现与GTdR Cdc 42相互作用。WASp似乎提供了Cdc 42和肌动蛋白细胞骨架之间的联系,也许解释了疾病背后的细胞缺陷。
The gene responsible for Wiskott-Aldrich syndrome, a disease affecting platelets and lymphocytes, has been cloned and its protein product (WASp) found to interact with the GTPase Cdc42. WASp seems to provide a link between Cdc42 and the actin cytoskeleton, perhaps explaining the cellular defects underlying the disease.