A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity

A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity Publisher Pubmed

Beziat V^{1, 2} ; Li J³ ; Lin JX⁴ ; Ma CS^{5, 6} ; Li P⁴ ; Bousfiha A⁷ ; Pellier I⁸ ; Zoghi S^{9, 10, 11} ; Baris S¹² ; Keles S¹³ ; Gray P^{14, 15} ; Du N⁴ ; Wang Y^{1, 2} ; Zerbib Y^{1, 2} Show All Authors

Beziat V^{1, 2}
Li J³
Lin JX⁴
Ma CS^{5, 6}
Li P⁴
Bousfiha A⁷
Pellier I⁸
Zoghi S^{9, 10, 11}
Baris S¹²
Keles S¹³
Gray P^{14, 15}
Du N⁴
Wang Y^{1, 2}
Zerbib Y^{1, 2}
Levy R^{1, 2}
Leclercq T^{1, 2}
About F^{1, 2}
Lim AI^{16, 17}
Rao G⁵
Payne K⁵
Pelham SJ^{5, 6}
Avery DT⁵
Deenick EK^{5, 6}
Pillay B^{5, 6}
Chou J^{18, 19}
Guery R^{1, 2, 20}
Belkadi A^{1, 2}
Guerin A^{1, 2}
Migaud M^{1, 2}
Rattina V^{1, 2}
Ailal F⁷
Benhsaien I⁷
Bouaziz M^{1, 2}
Habib T²¹
Chaussabel D²¹
Marr N²¹
Elbenna J²²
Grimbacher B²³
Wargon O²⁴
Bustamante J^{1, 2, 3, 25}
Boisson B^{1, 2, 3}
Mullerfleckenstein I²⁶
Fleckenstein B²⁶
Chandesris MO^{27, 28}
Titeux M^{2, 29}
Fraitag S³⁰
Alyanakian MA³¹
Leruezville M^{32, 33}
Picard C^{2, 25, 33, 34}
Meyts I³⁵
Di Santo JP^{16, 17}
Hovnanian A^{2, 29, 36}
Somer A³⁷
Ozen A¹²
Rezaei N^{9, 10, 11}
Chatila TA^{18, 19}
Abel L^{1, 2, 3}
Leonard WJ⁴
Tangye SG^{5, 6}
Puel A^{1, 2, 3}
Casanova JL^{1, 2, 3, 34, 38}

Source: Science Immunology Published:2018

Abstract

Heterozygosity for human signal transducer and activator of transcription 3 (STAT3) dominant-negative (DN) mutations underlies an autosomal dominant form of hyper–immunoglobulin E syndrome (HIES). We describe patients with an autosomal recessive form of HIES due to loss-of-function mutations of a previously uncharacterized gene, ZNF341. ZNF341 is a transcription factor that resides in the nucleus, where it binds a specific DNA motif present in various genes, including the STAT3 promoter. The patients’ cells have low basal levels of STAT3 mRNA and protein. The autoinduction of STAT3 production, activation, and function by STAT3-activating cytokines is strongly impaired. Like patients with STAT3 DN mutations, ZNF341-deficient patients lack T helper 17 (TH17) cells, have an excess of TH2 cells, and have low memory B cells due to the tight dependence of STAT3 activity on ZNF341 in lymphocytes. Their milder extra-hematopoietic manifestations and stronger inflammatory responses reflect the lower ZNF341 dependence of STAT3 activity in other cell types. Human ZNF341 is essential for the STAT3 transcription–dependent autoinduction and sustained activity of STAT3. Copyright © 2018 The Authors, some rights reserved.

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Style	Citing Format
MLA	Beziat V, et al.. "A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity." Science Immunology, vol. 3, no. 24, 2018, pp. -.
APA	Beziat V, Li J, Lin JX, Ma CS, Li P, Bousfiha A, Pellier I, Zoghi S, Baris S, Keles S, Gray P, Du N, Wang Y, Zerbib Y, Levy R, Leclercq T, About F, Lim AI, Rao G, ... Casanova JL (2018). A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity. Science Immunology, 3(24), -.
Chicago	Beziat V, Li J, Lin JX, Ma CS, Li P, Bousfiha A, Pellier I, et al.. "A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity." Science Immunology 3, no. 24 (2018): -.
Harvard	Beziat V et al. (2018) 'A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity', Science Immunology, 3(24), pp. -.
Vancouver	Beziat V, Li J, Lin JX, Ma CS, Li P, Bousfiha A, et al.. A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity. Science Immunology. 2018;3(24):-.
BibTex	@article{ author = {Beziat V and Li J and Lin JX and Ma CS and Li P and Bousfiha A and Pellier I and Zoghi S and Baris S and Keles S and Gray P and Du N and Wang Y and Zerbib Y and Levy R and Leclercq T and About F and Lim AI and Rao G and Payne K and Pelham SJ and Avery DT and Deenick EK and Pillay B and Chou J and Guery R and Belkadi A and Guerin A and Migaud M and Rattina V and Ailal F and Benhsaien I and Bouaziz M and Habib T and Chaussabel D and Marr N and Elbenna J and Grimbacher B and Wargon O and Bustamante J and Boisson B and Mullerfleckenstein I and Fleckenstein B and Chandesris MO and Titeux M and Fraitag S and Alyanakian MA and Leruezville M and Picard C and Meyts I and Di Santo JP and Hovnanian A and Somer A and Ozen A and Rezaei N and Chatila TA and Abel L and Leonard WJ and Tangye SG and Puel A and Casanova JL}, title = {A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity}, journal = {Science Immunology}, volume = {3}, number = {24}, pages = {-}, year = {2018} }
RIS	TY - JOUR AU - Beziat V AU - Li J AU - Lin JX AU - Ma CS AU - Li P AU - Bousfiha A AU - Pellier I AU - Zoghi S AU - Baris S AU - Keles S AU - Gray P AU - Du N AU - Wang Y AU - Zerbib Y AU - Levy R AU - Leclercq T AU - About F AU - Lim AI AU - Rao G AU - Payne K AU - Pelham SJ AU - Avery DT AU - Deenick EK AU - Pillay B AU - Chou J AU - Guery R AU - Belkadi A AU - Guerin A AU - Migaud M AU - Rattina V AU - Ailal F AU - Benhsaien I AU - Bouaziz M AU - Habib T AU - Chaussabel D AU - Marr N AU - Elbenna J AU - Grimbacher B AU - Wargon O AU - Bustamante J AU - Boisson B AU - Mullerfleckenstein I AU - Fleckenstein B AU - Chandesris MO AU - Titeux M AU - Fraitag S AU - Alyanakian MA AU - Leruezville M AU - Picard C AU - Meyts I AU - Di Santo JP AU - Hovnanian A AU - Somer A AU - Ozen A AU - Rezaei N AU - Chatila TA AU - Abel L AU - Leonard WJ AU - Tangye SG AU - Puel A AU - Casanova JL TI - A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity JO - Science Immunology VL - 3 IS - 24 SP - EP - PY - 2018 ER -

Style

Citing Format

MLA

Beziat V, et al.. "A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity." Science Immunology, vol. 3, no. 24, 2018, pp. -.

APA

Beziat V, Li J, Lin JX, Ma CS, Li P, Bousfiha A, Pellier I, Zoghi S, Baris S, Keles S, Gray P, Du N, Wang Y, Zerbib Y, Levy R, Leclercq T, About F, Lim AI, Rao G, ... Casanova JL (2018). A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity. Science Immunology, 3(24), -.

Chicago

Beziat V, Li J, Lin JX, Ma CS, Li P, Bousfiha A, Pellier I, et al.. "A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity." Science Immunology 3, no. 24 (2018): -.

Harvard

Beziat V et al. (2018) 'A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity', Science Immunology, 3(24), pp. -.

Vancouver

Beziat V, Li J, Lin JX, Ma CS, Li P, Bousfiha A, et al.. A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity. Science Immunology. 2018;3(24):-.

BibTex

@article{ author = {Beziat V and Li J and Lin JX and Ma CS and Li P and Bousfiha A and Pellier I and Zoghi S and Baris S and Keles S and Gray P and Du N and Wang Y and Zerbib Y and Levy R and Leclercq T and About F and Lim AI and Rao G and Payne K and Pelham SJ and Avery DT and Deenick EK and Pillay B and Chou J and Guery R and Belkadi A and Guerin A and Migaud M and Rattina V and Ailal F and Benhsaien I and Bouaziz M and Habib T and Chaussabel D and Marr N and Elbenna J and Grimbacher B and Wargon O and Bustamante J and Boisson B and Mullerfleckenstein I and Fleckenstein B and Chandesris MO and Titeux M and Fraitag S and Alyanakian MA and Leruezville M and Picard C and Meyts I and Di Santo JP and Hovnanian A and Somer A and Ozen A and Rezaei N and Chatila TA and Abel L and Leonard WJ and Tangye SG and Puel A and Casanova JL}, title = {A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity}, journal = {Science Immunology}, volume = {3}, number = {24}, pages = {-}, year = {2018} }

RIS

TY - JOUR
AU - Beziat V
AU - Li J
AU - Lin JX
AU - Ma CS
AU - Li P
AU - Bousfiha A
AU - Pellier I
AU - Zoghi S
AU - Baris S
AU - Keles S
AU - Gray P
AU - Du N
AU - Wang Y
AU - Zerbib Y
AU - Levy R
AU - Leclercq T
AU - About F
AU - Lim AI
AU - Rao G
AU - Payne K
AU - Pelham SJ
AU - Avery DT
AU - Deenick EK
AU - Pillay B
AU - Chou J
AU - Guery R
AU - Belkadi A
AU - Guerin A
AU - Migaud M
AU - Rattina V
AU - Ailal F
AU - Benhsaien I
AU - Bouaziz M
AU - Habib T
AU - Chaussabel D
AU - Marr N
AU - Elbenna J
AU - Grimbacher B
AU - Wargon O
AU - Bustamante J
AU - Boisson B
AU - Mullerfleckenstein I
AU - Fleckenstein B
AU - Chandesris MO
AU - Titeux M
AU - Fraitag S
AU - Alyanakian MA
AU - Leruezville M
AU - Picard C
AU - Meyts I
AU - Di Santo JP
AU - Hovnanian A
AU - Somer A
AU - Ozen A
AU - Rezaei N
AU - Chatila TA
AU - Abel L
AU - Leonard WJ
AU - Tangye SG
AU - Puel A
AU - Casanova JL
TI - A Recessive Form of Hyper-Ige Syndrome by Disruption of Znf341-Dependent Stat3 Transcription and Activity
JO - Science Immunology
VL - 3
IS - 24
SP -
EP -
PY - 2018
ER -

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