Abstract：
:DOCK8 immunodeficiency syndrome (DIDS) is a progressive combined immunodeficiency that can be distinguished from other combined immunodeficiencies or hyperimmunoglobulinemia E syndromes in featuring (a) profound susceptibility to virus infections of the skin, with associated skin cancers, and (b) severe food allergies. The DOCK8 locus has many repetitive sequence elements that predispose to the generation of large germline deletions as well as recombination-mediated somatic DNA repair. Residual DOCK8 protein contributes to the variable disease phenotype. The severe virus infections of the skin, and probably also VZV-associated vasculopathy, reflect an important function of DOCK8, which is normally required to maintain lymphocyte shape integrity as the cells migrate through dense tissues. Loss of DOCK8 also causes immune deficits through other mechanisms including a milder generalized cell survival defect and skewing of T helper cell subsets. Recent work has uncovered the roles for DOCK8 in dendritic cell responses that can also help explain the virus susceptibility, as well as in regulatory T cells that might help explain autoimmunity in a minority of patients. Fortunately, hematopoietic stem cell transplantation cures the eczema and infection susceptibility of DIDS, but not necessarily the other disease manifestations including food allergies.
journal_name
Immunol Revjournal_title
Immunological reviewsauthors
Su HC,Jing H,Angelus P,Freeman AFdoi
10.1111/imr.12723subject
Has Abstractpub_date
2019-01-01 00:00:00pages
9-19issue
1eissn
0105-2896issn
1600-065Xjournal_volume
287pub_type
杂志文章,评审abstract：:We first identified the RANTES chemokine as part of a search for genes expressed by T lymphocytes "late", 3-5 days, after T-cell activation. The kinetics of expression of RANTES and a small number of other genes are unusual and the mechanism of such delayed expression is unknown. In order to uncover a mechanism for su...
journal_title：Immunological reviews
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doi：10.1034/j.1600-065x.2000.17610.x
更新日期：2000-10-01 00:00:00
abstract：:T-cell development primarily occurs in the thymus and involves in the interactions of many important transcription factors. Until recently, no single transcription factor has been identified to be essential for T-cell lineage commitment or maintenance of T-cell identity. Recent studies have now identified the zinc fin...
journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1111/j.1600-065X.2010.00953.x
更新日期：2010-11-01 00:00:00
abstract：:Schistosomiasis is a serious global helminthic disease, in which the main immunopathology consists of a granulomatous and fibrosing reaction against tissue-trapped parasite eggs. The severity of this inflammatory process, the product of a CD4(+) T-cell-mediated immune response against parasite egg antigens, is, howeve...
journal_title：Immunological reviews
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更新日期：2004-10-01 00:00:00
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journal_title：Immunological reviews
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更新日期：1998-04-01 00:00:00
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journal_title：Immunological reviews
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更新日期：1997-02-01 00:00:00
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journal_title：Immunological reviews
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更新日期：1998-08-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1111/imr.12552
更新日期：2017-07-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
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更新日期：1991-04-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
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更新日期：1999-02-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
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更新日期：2011-03-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
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更新日期：1989-08-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1111/j.1600-065X.2008.00631.x
更新日期：2008-06-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1111/j.1600-065x.1985.tb01148.x
更新日期：1985-10-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1034/j.1600-065x.2001.1810116.x
更新日期：2001-06-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1111/j.0105-2896.2006.00348.x
更新日期：2006-02-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1111/imr.12446
更新日期：2016-09-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1111/j.1600-065x.1997.tb00972.x
更新日期：1997-06-01 00:00:00
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pub_type： 杂志文章,评审
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更新日期：2012-11-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1111/j.1600-065x.1998.tb01260.x
更新日期：1998-12-01 00:00:00
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journal_title：Immunological reviews
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更新日期：2015-03-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
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更新日期：2006-02-01 00:00:00
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journal_title：Immunological reviews
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更新日期：2009-11-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1034/j.1600-065x.2003.00025.x
更新日期：2003-02-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1046/j.1600-065x.2003.00088.x
更新日期：2003-12-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1111/j.0105-2896.2005.00310.x
更新日期：2005-10-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1111/j.1600-065x.1995.tb00075.x
更新日期：1995-06-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1111/j.1600-065X.2011.01045.x
更新日期：2011-09-01 00:00:00
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journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1111/j.1600-065X.2012.01165.x
更新日期：2012-11-01 00:00:00
abstract：:The activities of the immune system in repairing tissue injury and combating pathogens were long thought to be independent of the nervous system. However, a major regulatory role of immunomodulatory molecules released locally or systemically by the neuroendocrine system has recently emerged. A number of observations a...
journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1111/imr.12707
更新日期：2018-11-01 00:00:00
abstract：:The mammalian Dok protein family has seven members (Dok-1-Dok-7). The Dok proteins share structural similarities characterized by the NH2-terminal pleckstrin homology and phosphotyrosine-binding domains followed by SH2 target motifs in the COOH-terminal moiety, indicating an adapter function. Indeed, Dok-1 was origina...
journal_title：Immunological reviews
pub_type： 杂志文章,评审
doi：10.1111/j.1600-065X.2009.00844.x
更新日期：2009-11-01 00:00:00
