免疫学
免疫失调
Wiskott–Aldrich综合征蛋白
Wiskott-Aldrich综合征
生物
免疫系统
单倍率不足
促炎细胞因子
异位表达
细胞生物学
癌症研究
炎症
肌动蛋白细胞骨架
表型
遗传学
细胞骨架
细胞
基因
作者
Jana Block,Christina Rashkova,Irinka Castanon,Samaneh Zoghi,Jessica Platon,Rico Chandra Ardy,M. Fujiwara,Beatriz Chaves,Rouven Schoppmeyer,Caspar I. van der Made,Raúl Jiménez Heredia,Frederike L. Harms,Samin Alavi,Laia Alsina,Paula Sánchez Moreno,Rainiero Ávila Polo,Rocío Cabrera‐Pérez,Sevgi Köstel Bal,Laurène Pfajfer,Bernhard Ransmayr
标识
DOI:10.1056/nejmoa2210054
摘要
Germline hemizygous loss-of-function mutations affecting the actin regulator DOCK11 were shown to cause a previously unknown inborn error of hematopoiesis and immunity characterized by severe immune dysregulation and systemic inflammation, recurrent infections, and anemia. (Funded by the European Research Council and others.).
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