Science Immunology

Supplementary Materials

Supplementary Material for:

ZNF341 controls STAT3 expression and thereby immunocompetence

Stefanie Frey-Jakobs, Julia M. Hartberger, Manfred Fliegauf, Claudia Bossen, Magdalena L. Wehmeyer, Johanna C. Neubauer, Alla Bulashevska, Michele Proietti, Philipp Fröbel, Christina Nöltner, Linlin Yang, Jessica Rojas-Restrepo, Niko Langer, Sandra Winzer, Karin R. Engelhardt, Cristina Glocker, Dietmar Pfeifer, Adi Klein, Alejandro A. Schäffer, Irina Lagovsky, Idit Lachover-Roth, Vivien Béziat, Anne Puel, Jean-Laurent Casanova, Bernhard Fleckenstein, Stephan Weidinger, Sara S. Kilic, Ben-Zion Garty, Amos Etzioni, Bodo Grimbacher*

*Corresponding authors. Email: bodo.grimbacher{at}

Published 15 June 2018, Sci. Immunol. 3, eaat4941 (2018)
DOI: 10.1126/sciimmunol.aat4941

This PDF file includes:

  • Section S1. Case reports for families A to D
  • Section S2. Defective B cell development and TH17 cell differentiation in HIES patients with ZNF341 nonsense mutations
  • Section S3. Exclusion of mutations in STAT3 in the etiology of the HIES phenotype in family A
  • Section S4. Identification of ZNF341 as an AR-HIES–associated gene in a linkage region on chromosome 20 by WES
  • Section S5. Expression of various ZNF341 mRNA variants in multiple cell lines and PBMCs
  • Section S6. Supplementary Materials and Methods
  • Fig. S1. B and T cell subsets in patient-derived and HD PBMCs.
  • Fig. S2. Ubiquitous expression of ZNF341 mRNA in various cell types.
  • Fig. S3. No association of the genomic region 20q11.22 spanning the ZNF341 locus with atopy.
  • Fig. S4. Ectopic expression of ZNF341 variants in HEK293T cells.
  • Fig. S5. Ectopically expressed wt ZNF341 binds to the STAT3 promoter leading to transcriptional activation.
  • Fig. S6. Occupancy by ZNF341 across the STAT3 promoter as determined by ChIP.
  • Fig. S7. ZNF341 R386* shows reduced binding to the STAT3 promoter.
  • Fig. S8. Flow cytometry gating strategy for in vitro TH17 cell differentiation assay.
  • Fig. S9. Flow cytometry gating strategy for Y705 phosphorylation of STAT3.
  • Fig. S10. Flow cytometry gating strategy for immune phenotyping of PBMCs.
  • Fig. S11. Flow cytometry gating strategy for IL-22+ T cells.
  • Table S1. Clinical and immunological phenotype of HIES patients with ZNF341 mutations.
  • Table S2. ZNF341 encodes three protein coding isoforms.
  • Table S3. Transcriptome analysis of patient A.II.1 with gene-specific fold changes in comparison to healthy sibling A.II.5.
  • References (47–53)

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Other Supplementary Material for this manuscript includes the following:

  • Table S4 (Microsoft Excel format). Raw data.

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