Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Comment
. 2018 Jun 15;3(24):eaat9779.
doi: 10.1126/sciimmunol.aat9779.

Who regulates whom: ZNF341 is an additional player in the STAT3/TH17 song

Affiliations
Comment

Who regulates whom: ZNF341 is an additional player in the STAT3/TH17 song

Avery August. Sci Immunol. .

Abstract

Deficiency of ZNF341, a transcription factor featuring 12 Cys2His2 zinc fingers that regulates the expression and autoinduction of STAT3 (signal transducer and activator of transcription 3), results in hyper-immunoglobulin E syndrome and defective T helper 17 cell differentiation in humans.

PubMed Disclaimer

Conflict of interest statement

Conflict of Interest Disclosure

The author declares no competing financial interests.

Figures

Figure 1.
Figure 1.. ZNF341, STAT3 and Th17 differentiation, a more complex song.
A) Th17 differentiation requires TCR signals (not shown) along with cytokines IL-6, TGF-β, and/or IL-1β. Signaling through the IL-6 receptor triggers activation of JAK2, which phosphorylates STAT3, driving its dimerization and nuclear localization. STAT3 cooperates with other transcription factors such as SMADs to drive expression of critical genes required for Th17 differentiation. ZNF341 regulates STAT3 expression and its autoinduction during Th17 differentiation. (B) Key structural motifs within ZNF341 and the location of mutations in HIES patients identified by Beziat et al and Frey-Jacobs et al,.

Comment on

  • A recessive form of hyper-IgE syndrome by disruption of ZNF341-dependent STAT3 transcription and activity.
    Béziat 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, Lévy R, Leclercq T, About F, Lim AI, Rao G, Payne K, Pelham SJ, Avery DT, Deenick EK, Pillay B, Chou J, Guery R, Belkadi A, Guérin A, Migaud M, Rattina V, Ailal F, Benhsaien I, Bouaziz M, Habib T, Chaussabel D, Marr N, El-Benna J, Grimbacher B, Wargon O, Bustamante J, Boisson B, Müller-Fleckenstein I, Fleckenstein B, Chandesris MO, Titeux M, Fraitag S, Alyanakian MA, Leruez-Ville M, Picard C, Meyts I, Di Santo JP, Hovnanian A, Somer A, Ozen A, Rezaei N, Chatila TA, Abel L, Leonard WJ, Tangye SG, Puel A, Casanova JL. Béziat V, et al. Sci Immunol. 2018 Jun 15;3(24):eaat4956. doi: 10.1126/sciimmunol.aat4956. Sci Immunol. 2018. PMID: 29907691 Free PMC article.

Similar articles

  • A recessive form of hyper-IgE syndrome by disruption of ZNF341-dependent STAT3 transcription and activity.
    Béziat 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, Lévy R, Leclercq T, About F, Lim AI, Rao G, Payne K, Pelham SJ, Avery DT, Deenick EK, Pillay B, Chou J, Guery R, Belkadi A, Guérin A, Migaud M, Rattina V, Ailal F, Benhsaien I, Bouaziz M, Habib T, Chaussabel D, Marr N, El-Benna J, Grimbacher B, Wargon O, Bustamante J, Boisson B, Müller-Fleckenstein I, Fleckenstein B, Chandesris MO, Titeux M, Fraitag S, Alyanakian MA, Leruez-Ville M, Picard C, Meyts I, Di Santo JP, Hovnanian A, Somer A, Ozen A, Rezaei N, Chatila TA, Abel L, Leonard WJ, Tangye SG, Puel A, Casanova JL. Béziat V, et al. Sci Immunol. 2018 Jun 15;3(24):eaat4956. doi: 10.1126/sciimmunol.aat4956. Sci Immunol. 2018. PMID: 29907691 Free PMC article.
  • ZNF341 controls STAT3 expression and thereby immunocompetence.
    Frey-Jakobs S, Hartberger JM, Fliegauf M, Bossen C, Wehmeyer ML, Neubauer JC, Bulashevska A, Proietti M, Fröbel P, Nöltner C, Yang L, Rojas-Restrepo J, Langer N, Winzer S, Engelhardt KR, Glocker C, Pfeifer D, Klein A, Schäffer AA, Lagovsky I, Lachover-Roth I, Béziat V, Puel A, Casanova JL, Fleckenstein B, Weidinger S, Kilic SS, Garty BZ, Etzioni A, Grimbacher B. Frey-Jakobs S, et al. Sci Immunol. 2018 Jun 15;3(24):eaat4941. doi: 10.1126/sciimmunol.aat4941. Sci Immunol. 2018. PMID: 29907690 Free PMC article.
  • Inherited human ZNF341 deficiency.
    Béziat V, Fieschi C, Momenilandi M, Migaud M, Belaid B, Djidjik R, Puel A. Béziat V, et al. Curr Opin Immunol. 2023 Jun;82:102326. doi: 10.1016/j.coi.2023.102326. Epub 2023 Apr 18. Curr Opin Immunol. 2023. PMID: 37080116 Free PMC article. Review.
  • Dedicator of cytokinesis 8 regulates signal transducer and activator of transcription 3 activation and promotes TH17 cell differentiation.
    Keles S, Charbonnier LM, Kabaleeswaran V, Reisli I, Genel F, Gulez N, Al-Herz W, Ramesh N, Perez-Atayde A, Karaca NE, Kutukculer N, Wu H, Geha RS, Chatila TA. Keles S, et al. J Allergy Clin Immunol. 2016 Nov;138(5):1384-1394.e2. doi: 10.1016/j.jaci.2016.04.023. Epub 2016 May 24. J Allergy Clin Immunol. 2016. PMID: 27350570 Free PMC article.
  • The signal transducer and activator of transcription 3 at the center of the causative gene network of the hyper-IgE syndrome.
    Minegishi Y. Minegishi Y. Curr Opin Immunol. 2023 Feb;80:102264. doi: 10.1016/j.coi.2022.102264. Epub 2022 Nov 24. Curr Opin Immunol. 2023. PMID: 36435159 Review.

Cited by

References

    1. Béziat V, Li J, Lin J-X, Ma CS, Li P, Bousfiha A, Pellier I, Zoghi S, Baris S, Keles S, Gray P, Du N, Wang Y, Zerbib Y, Lévy R, Leclercq T, About F, Ing Lim A, Rao G, Payne K, Pelham SJ, Avery DT, Deenick EK, Pillay B, Chou J, Guery R, Belkadi A, Guérin A, Migaud M, Rattina V, Ailal F, Benhsaien I, Bouaziz M, Tanwir H, Chaussabel D, Marr N, El Benna J, Grimbacher B, Wargon O, Bustamante JC, Boisson B, Müller-Fleckenstein I, Fleckenstein B, Chandesris M-O, Titeux M, Fraitag S, Alyanakian M-A, Leruez-Ville M, Picard C, Meyts I, Di Santo JP, Hovnanian A, Somer A, Ozen A, Rezaei N, Chatila T, Abel L, Leonard WJ, Tangye SG, Puel A, Casanova J-L, A recessive form of Hyper IgE Syndrome by disruption of ZNF341-dependent STAT3 transcription and activity. Sci. Immunol 3, eaat4956 (2018). - PMC - PubMed
    1. Frey-Jakobs S, Hartberger JM, Fliegauf M, Bossen C, Wehmeyer ML, Neubauer JC, Bulashevska A, Proietti M, Fröbel P, Nöltner C, Yang L, Rojas-Restrepo J, Langer N, Winzer S, Engelhardt KR, Glocker C, Pfeifer D, Klein A, Schäffer AA, Lagovsky I, Lachover-Roth I, Béziat V, Puel A, Casanova J-L, Fleckenstein B, Weidinger S, Kilic SS, Garty B-Z, Etzioni A, Grimbacher B, ZNF341 controls STAT3 expression and thereby immunocompetence. Sci. Immunol 3, eaat4941 (2018). - PMC - PubMed
    1. Mogensen TH “Primary Immunodeficiencies with Elevated IgE”. Int Rev Immunol 35: 39–56, (2016) - PubMed
    1. Hillmer EJ, Zhang H, Li HS, Watowich SS “STAT3 signaling in immunity”. Cytokine Growth Factor Rev 31:1–15 (2016) - PMC - PubMed
    1. Keles S, Charbonnier LM, Kabaleeswaran V, Reisli I, Genel F, Gulez N, Al-Herz W, Ramesh N, Perez-Atayde A, Karaca NE, Kutukculer N, Wu H, Geha RS, Chatila TA “Dedicator of cytokinesis 8 regulates signal transducer and activator of transcription 3 activation and promotes TH17 cell differentiation” J Allergy Clin Immunol 138:1384–1394.e2 (2016) - PMC - PubMed

Publication types

Substances