Defective Toll-Like Receptors Driven B Cell Response in Hyper IgE Syndrome Patients With STAT3 Mutations

Autosomal dominant hyper-IgE syndrome (AD-HIES) is a rare inherited primary immunodeficient disease (PIDs), which is caused by STAT3 gene mutations. Previous studies indicated a defective Toll-like receptor (TLR) 9-induced B cell response in AD-HIES patients, including proliferation, and IgG product...

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Autores principales:	Ruolan Gong, Jing Wu, Yingying Jin, Tongxin Chen
Formato:	article
Lenguaje:	EN
Publicado:	Frontiers Media S.A. 2021
Materias:	TLR7 TLR9 stat3 hyper IgE syndrome (HIES) B cell function Pediatrics RJ1-570
Acceso en línea:	https://doaj.org/article/36ab027f2fb744b5b0561c03d49ca4ed
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spelling	oai:doaj.org-article:36ab027f2fb744b5b0561c03d49ca4ed2021-11-05T16:50:42ZDefective Toll-Like Receptors Driven B Cell Response in Hyper IgE Syndrome Patients With STAT3 Mutations2296-236010.3389/fped.2021.738799https://doaj.org/article/36ab027f2fb744b5b0561c03d49ca4ed2021-11-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fped.2021.738799/fullhttps://doaj.org/toc/2296-2360Autosomal dominant hyper-IgE syndrome (AD-HIES) is a rare inherited primary immunodeficient disease (PIDs), which is caused by STAT3 gene mutations. Previous studies indicated a defective Toll-like receptor (TLR) 9-induced B cell response in AD-HIES patients, including proliferation, and IgG production. However, the other TLRs-mediated B cell responses in AD-HIES patients were not fully elucidated. In this study, we systematically studied the B cell response to TLRs signaling pathways in AD-HIES patients, including proliferation, activation, apoptosis, cytokine, and immunoglobulin production. Our results showed that the TLRs-induced B cell proliferation and activation was significantly impaired in AD-HIES patients. Besides, AD-HIES patients had defects in TLRs-induced B cell class switch, as well as IgG/IgM secretion and IL-10 production in B cells. Taken together, we first systematically reported the deficiency of TLRs driven B cell response in AD-HIES patients, which help to have a better understanding of the pathology of AD-HIES.Ruolan GongJing WuJing WuYingying JinYingying JinTongxin ChenTongxin ChenTongxin ChenFrontiers Media S.A.articleTLR7TLR9stat3hyper IgE syndrome (HIES)B cell functionPediatricsRJ1-570ENFrontiers in Pediatrics, Vol 9 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	TLR7 TLR9 stat3 hyper IgE syndrome (HIES) B cell function Pediatrics RJ1-570
spellingShingle	TLR7 TLR9 stat3 hyper IgE syndrome (HIES) B cell function Pediatrics RJ1-570 Ruolan Gong Jing Wu Jing Wu Yingying Jin Yingying Jin Tongxin Chen Tongxin Chen Tongxin Chen Defective Toll-Like Receptors Driven B Cell Response in Hyper IgE Syndrome Patients With STAT3 Mutations
description	Autosomal dominant hyper-IgE syndrome (AD-HIES) is a rare inherited primary immunodeficient disease (PIDs), which is caused by STAT3 gene mutations. Previous studies indicated a defective Toll-like receptor (TLR) 9-induced B cell response in AD-HIES patients, including proliferation, and IgG production. However, the other TLRs-mediated B cell responses in AD-HIES patients were not fully elucidated. In this study, we systematically studied the B cell response to TLRs signaling pathways in AD-HIES patients, including proliferation, activation, apoptosis, cytokine, and immunoglobulin production. Our results showed that the TLRs-induced B cell proliferation and activation was significantly impaired in AD-HIES patients. Besides, AD-HIES patients had defects in TLRs-induced B cell class switch, as well as IgG/IgM secretion and IL-10 production in B cells. Taken together, we first systematically reported the deficiency of TLRs driven B cell response in AD-HIES patients, which help to have a better understanding of the pathology of AD-HIES.
format	article
author	Ruolan Gong Jing Wu Jing Wu Yingying Jin Yingying Jin Tongxin Chen Tongxin Chen Tongxin Chen
author_facet	Ruolan Gong Jing Wu Jing Wu Yingying Jin Yingying Jin Tongxin Chen Tongxin Chen Tongxin Chen
author_sort	Ruolan Gong
title	Defective Toll-Like Receptors Driven B Cell Response in Hyper IgE Syndrome Patients With STAT3 Mutations
title_short	Defective Toll-Like Receptors Driven B Cell Response in Hyper IgE Syndrome Patients With STAT3 Mutations
title_full	Defective Toll-Like Receptors Driven B Cell Response in Hyper IgE Syndrome Patients With STAT3 Mutations
title_fullStr	Defective Toll-Like Receptors Driven B Cell Response in Hyper IgE Syndrome Patients With STAT3 Mutations
title_full_unstemmed	Defective Toll-Like Receptors Driven B Cell Response in Hyper IgE Syndrome Patients With STAT3 Mutations
title_sort	defective toll-like receptors driven b cell response in hyper ige syndrome patients with stat3 mutations
publisher	Frontiers Media S.A.
publishDate	2021
url	https://doaj.org/article/36ab027f2fb744b5b0561c03d49ca4ed
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Defective Toll-Like Receptors Driven B Cell Response in Hyper IgE Syndrome Patients With STAT3 Mutations

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