Abstract：
:Mutations of the Wiskott-Aldrich Syndrome Protein (WASP) are responsible for classic Wiskott-Aldrich Syndrome (WAS), X-linked thrombocytopenia (XLT), and in rare instances congenital X-linked neutropenia (XLN). WASP is a regulator of actin polymerization in hematopoietic cells with well-defined functional domains that are involved in cell signaling and cell locomotion, immune synapse formation, and apoptosis. Mutations of WASP are located throughout the gene and either inhibit or disregulate normal WASP function. Analysis of a large patient population demonstrates a strong phenotype-genotype correlation. Classic WAS occurs when WASP is absent, XLT when mutated WASP is expressed and XLN when missense mutations occur in the Cdc42-binding site. However, because there are exceptions to this rule it is difficult to predict the long-term prognosis of a given affected boy solely based on the analysis of WASP expression.
journal_name
Immunol Resjournal_title
Immunologic researchauthors
Ochs HDdoi
10.1007/s12026-008-8084-3subject
Has Abstractpub_date
2009-01-01 00:00:00pages
84-8issue
1-3eissn
0257-277Xissn
1559-0755journal_volume
44pub_type
杂志文章,评审abstract：:Thermal injury, as well as other forms of severe trauma, induces simultaneous hyper- and anti-inflammatory response. While data about decreased number and responsiveness of T lymphocytes are largely consistent, reports concerning granulocytes following trauma are contradictory. Contrary to the evidence on the increase...
journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1007/s12026-012-8280-z
更新日期：2012-04-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,meta分析
doi：10.1007/s12026-019-09102-x
更新日期：2019-10-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章
doi：10.1007/s12026-016-8876-9
更新日期：2016-12-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1007/s12026-007-0070-7
更新日期：2007-01-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1007/BF02918253
更新日期：1996-01-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1007/BF02786471
更新日期：1999-01-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1007/s12026-009-8093-x
更新日期：2009-12-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章
doi：10.1007/s12026-020-09115-x
更新日期：2020-02-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章
doi：10.1007/s12026-016-8785-y
更新日期：2016-08-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1007/s12026-018-9051-2
更新日期：2018-12-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章
doi：10.1007/s12026-011-8206-1
更新日期：2011-08-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1385/IR:27:1:85
更新日期：2003-01-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章
doi：10.1007/s12026-008-8024-2
更新日期：2009-01-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1007/BF02786399
更新日期：1997-01-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章
doi：10.1007/s12026-017-8900-8
更新日期：2017-06-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章
doi：10.1007/s12026-020-09123-x
更新日期：2020-02-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章
doi：10.1007/BF02935511
更新日期：1989-01-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1007/s12026-008-8078-1
更新日期：2009-01-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1385/IR:21:2-3:293
更新日期：2000-01-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1385/IR:29:1-3:055
更新日期：2004-01-01 00:00:00
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pub_type： 杂志文章
doi：10.1007/s12026-016-8867-x
更新日期：2017-02-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1007/BF02786433
更新日期：1998-01-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1385/IR:30:3:279
更新日期：2004-01-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章
doi：10.1007/s12026-011-8237-7
更新日期：2011-10-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章
doi：10.1007/s12026-020-09140-w
更新日期：2020-06-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1385/IR:34:3:177
更新日期：2006-01-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1007/s12026-012-8264-z
更新日期：2012-06-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章
doi：10.1007/BF02917584
更新日期：1986-01-01 00:00:00
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journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1007/s12026-017-8926-y
更新日期：2017-08-01 00:00:00
abstract：:Naive CD4 T cell activation is a complex process involving many steps. T cell receptor (TCR) signals, provided by interaction with peptide/MHC on antigen-presenting cells (APC), control many events associated with activation. The extent of TCR signaling and the magnitude of the T cell response is in turn controlled by...
journal_title：Immunologic research
pub_type： 杂志文章,评审
doi：10.1007/BF02918501
更新日期：1996-01-01 00:00:00
