An essential role for the Zn transporter ZIP7 in B cell development.

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ID: 56301
2019
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Abstract
Despite the known importance of zinc for human immunity, molecular insights into its roles have remained limited. Here we report a novel autosomal recessive disease characterized by absent B cells, agammaglobulinemia and early onset infections in five unrelated families. The immunodeficiency results from hypomorphic mutations of SLC39A7, which encodes the endoplasmic reticulum-to-cytoplasm zinc transporter ZIP7. Using CRISPR-Cas9 mutagenesis we have precisely modeled ZIP7 deficiency in mice. Homozygosity for a null allele caused embryonic death, but hypomorphic alleles reproduced the block in B cell development seen in patients. B cells from mutant mice exhibited a diminished concentration of cytoplasmic free zinc, increased phosphatase activity and decreased phosphorylation of signaling molecules downstream of the pre-B cell and B cell receptors. Our findings highlight a specific role for cytosolic Zn in modulating B cell receptor signal strength and positive selection.
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anzilotti2019annature Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors Consuelo Anzilotti, David J Swan, Bertrand Boisson, Mukta Deobagkar-Lele, Catarina Oliveira, Pauline Chabosseau, Karin R Engelhardt, Xijin Xu, Rui Chen, Luis Alvarez, Rolando Berlinguer-Palmini, Katherine R Bull, Eleanor Cawthorne, Adam P Cribbs, Tanya L Crockford, Tarana Singh Dang, Amy Fearn, Emma J Fenech, Sarah J de Jong, B Christoffer Lagerholm, Cindy S Ma, David Sims, Bert van den Berg, Yaobo Xu, Andrew J Cant, Gary Kleiner, T Ronan Leahy, M Teresa de la Morena, Jennifer M Puck, Ralph S Shapiro, Mirjam van der Burg, J Ross Chapman, John C Christianson, Benjamin Davies, John A McGrath, Stefan Przyborski, Mauro Santibanez Koref, Stuart G Tangye, Andreas Werner, Guy A Rutter, Sergi Padilla-Parra, Jean-Laurent Casanova, Richard J Cornall, Mary Ellen Conley, Sophie Hambleton
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Journal Nature Immunology
Year 2019
DOI
10.1038/s41590-018-0295-8
URL
Keywords
mercury trophic transfer baltic sea biomagnification coastal food web macrozoobenthos

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