IL-21 is the primary common γ chain-binding cytokine required for human B-cell differentiation in vivo

Blood. 2011 Dec 22;118(26):6824-35. doi: 10.1182/blood-2011-06-362533. Epub 2011 Oct 28.

Abstract

SCID resulting from mutations in IL2RG or JAK3 is characterized by lack of T and natural killer cells; B cells are present in normal number, but antibody responses are defective. Hematopoietic cell transplantation (HCT) is curative for SCID. However, B-cell dysfunction persists in a substantial proportion of patients. We hypothesized that impaired B-cell responses after HCT in IL2RG/JAK3 deficiency results from poor donor B-cell engraftment and defective γc-dependent cytokine signaling in host B cells. To test this, and to identify which γc cytokine(s) is critical for humoral immunity, we studied 28 transplanted patients with IL2RG/JAK3 deficiency. Lack of donor B-cell engraftment associated with persistent humoral dysfunction and significantly reduced memory B cells. B-cell proliferation induced by CD40L alone or together with CpG, anti-Ig, IL-4, IL-10, or IL-13 was comparable in healthy controls and in post-HCT SCID patients, irrespective of their chimerism status. However, in vitro stimulation with CD40L/IL-21 induced B-cell proliferation, plasmablast differentiation, and antibody secretion in patients with donor B cells, but not in patients with autologous B cells. These data imply that IL-21-mediated signaling is critical for long-lived humoral immunity and to restore antibody responses in IL2RG/JAK3-deficient patients after HCT. Furthermore, in vitro stimulation with CD40L/IL-21 can predict in vivo B-cell immunity in IL2RG/JAK3 SCID after transplantation.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • B-Lymphocytes / immunology*
  • B-Lymphocytes / metabolism
  • CD40 Ligand / immunology
  • CD40 Ligand / metabolism
  • Cell Differentiation / immunology*
  • Cells, Cultured
  • Flow Cytometry
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Immunoglobulin A / blood
  • Immunoglobulin A / immunology
  • Immunoglobulin M / blood
  • Immunoglobulin M / immunology
  • Immunologic Memory / immunology
  • Interleukin Receptor Common gamma Subunit / genetics
  • Interleukin Receptor Common gamma Subunit / immunology*
  • Interleukin Receptor Common gamma Subunit / metabolism
  • Interleukins / immunology*
  • Interleukins / metabolism
  • Janus Kinase 3 / genetics
  • Janus Kinase 3 / immunology
  • Janus Kinase 3 / metabolism
  • Mutation
  • Protein Binding
  • Severe Combined Immunodeficiency / genetics
  • Severe Combined Immunodeficiency / immunology
  • Severe Combined Immunodeficiency / metabolism
  • X-Linked Combined Immunodeficiency Diseases / genetics
  • X-Linked Combined Immunodeficiency Diseases / immunology
  • X-Linked Combined Immunodeficiency Diseases / metabolism

Substances

  • Immunoglobulin A
  • Immunoglobulin M
  • Interleukin Receptor Common gamma Subunit
  • Interleukins
  • CD40 Ligand
  • JAK3 protein, human
  • Janus Kinase 3
  • interleukin-21