Staphylococcus aureus and Hyper-IgE Syndrome.
Staphylococcus aureus and Hyper-IgE Syndrome.
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DOI：
10.3390/ijms21239152
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发表时间：
2020-12-01
影响因子：
5.6
通讯作者：
Liu GY
中科院分区：
文献类型：
--
作者：
Park B;Liu GY
Hyper-immunoglobulin E syndrome (HIES) is a primary immunodeficiency disease characterized by recurrent Staphylococcus aureus (S. aureus) infections, eczema, skeletal abnormalities and high titers of serum immunoglobulin E. Although the genetic basis of HIES was not known for almost a half century, HIES most frequently exhibits autosomal dominant trait that is transmitted with variable expressivity. Careful genetic studies in recent years identified dominant-negative mutations in human signal transducer and activator of transcription 3 (STAT3) gene as the cause of sporadic and dominant forms of HIES. The STAT3 mutations were localized to DNA-binding, SRC homology 2 (SH2) and transactivating domains and disrupted T helper 17 (TH17) cell differentiation and downstream expression of TH17 cytokines IL-17 and IL-22. Deficiency of IL-17 and IL-22 in turn is responsible for suboptimal expression of anti-staphylococcal host factors, such as neutrophil-recruiting chemokines and antimicrobial peptides, by human keratinocytes and bronchial epithelial cells. TH17 cytokines deficiency thereby explains the recurrent staphylococcal lung and skin infections of HIES patients.
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影响因子：
158.5
作者：
Grimbacher, B;Holland, SM;Puck, JM
通讯作者：
Puck, JM
影响因子：
--
作者：
Chen, Zhi;Tato, Cristina M.;O'Shea, John J.
通讯作者：
O'Shea, John J.
DOI：
10.1084/jem.20191804
发表时间：
2020-06-01
期刊：
The Journal of experimental medicine
影响因子：
--
作者：
Béziat V;Tavernier SJ;Chen YH;Ma CS;Materna M;Laurence A;Staal J;Aschenbrenner D;Roels L;Worley L;Claes K;Gartner L;Kohn LA;De Bruyne M;Schmitz-Abe K;Charbonnier LM;Keles S;Nammour J;Vladikine N;Maglorius Renkilaraj MRL;Seeleuthner Y;Migaud M;Rosain J;Jeljeli M;Boisson B;Van Braeckel E;Rosenfeld JA;Dai H;Burrage LC;Murdock DR;Lambrecht BN;Avettand-Fenoel V;Vogel TP;Undiagnosed Diseases Network;Esther CR;Haskologlu S;Dogu F;Ciznar P;Boutboul D;Ouachée-Chardin M;Amourette J;Lebras MN;Gauvain C;Tcherakian C;Ikinciogullari A;Beyaert R;Abel L;Milner JD;Grimbacher B;Couderc LJ;Butte MJ;Freeman AF;Catherinot É;Fieschi C;Chatila TA;Tangye SG;Uhlig HH;Haerynck F;Casanova JL;Puel A
通讯作者：
Puel A
DOI：
10.1016/s0924-8579(08)70002-5
发表时间：
2008-04-01
影响因子：
10.8
作者：
Gould, Ian M.
通讯作者：
Gould, Ian M.
影响因子：
4.8
作者：
Chen, Y;Thai, P;Wu, R
通讯作者：
Wu, R