Recent Advances in DOCK8 Immunodeficiency Syndrome

被引:31
作者
Zhang, Qian [1 ,2 ]
Jing, Huie [1 ]
Su, Helen C. [1 ]
机构
[1] NIAID, Host Def Lab, NIH, Bldg 10CRC,Room 5-3940,10 Ctr Dr,MSC 1456, Bethesda, MD 20892 USA
[2] Sidra Med & Res Ctr, Div Translat Med, Doha, Qatar
关键词
DOCK8; combined immunodeficiency; hyperimmunoglobulinemia E syndrome; tissue residen memory T cells (T-RM); cytothripsis; cutaneous virus infection; somatic reversion; genetics; eczema; food allergy; hematopoietic stem cell transplantation; STEM-CELL TRANSPLANTATION; T-CELLS; DOCK8-DEFICIENT PATIENTS; PROTECTIVE IMMUNITY; DEFICIENCY; DEDICATOR; SURVIVAL; PHENOTYPE; MAINTENANCE; MIGRATION;
D O I
10.1007/s10875-016-0296-z
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
Since the discovery of the genetic basis of DOCK8 immunodeficiency syndrome (DIDS) in 2009, several hundred patients worldwide have been reported, validating and extending the initial clinical descriptions. Importantly, the beneficial role of hematopoietic stem cell transplantation for this disease has emerged, providing impetus for improved diagnosis. Additionally, several groups have further elucidated the biological functions of DOCK8 in the immune system that help explain disease pathogenesis. Here, we summarize these recent developments.
引用
收藏
页码:441 / 449
页数:9
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