B-cell function in canine X-linked severe combined immunodeficiency

Brian J. Hartnett, Richard L. Somberg, Steven Krakowka, Hans D. Ochs, Harm Hogenesch, Peter F Moore, Kenneth I. Weinberg, Peter J. Felsburg

Research output: Contribution to journalArticlepeer-review

13 Scopus citations


Canine X-linked severe combined immunodeficiency (XSCID) is due to mutations in the common gamma (γc) subunit of the IL-2, IL-4, IL-7, IL-9 and IL-15 receptors and has a similar clinical phenotype to human XSCID. We have previously shown that the block in T-cell development is more profound in XSCID dogs than in genetically engineered γc-deficient mice. In this study we evaluated the B-cell function in XSCID dogs. In contrast to the marked decrease in peripheral B-cells in γc-deficient mice, XSCID dogs have increased proportions and numbers of peripheral B-cells as observed in XSCID boys. Canine XSCID B-cells do not proliferate following stimulation with the T-cell-dependent B-cell mitogen, pokeweed mitogen (PWM); however, they proliferate normally in response to the T-cell-independent B-cell mitogen, formalin-fixed, heat-killed Staphylococcus aureus. Canine XSCID B-cells are capable of producing IgM but are incapable of normal class-switching to IgG antibody production as demonstrated by in vitro stimulation with PWM and immunization with the T-cell-dependent antigen, bacteriophage ΦX174. Similar results have been reported for XSCID boys. Thus, it appears that γc-dependent cytokines have differing roles in human and canine B-cell development than in the mouse making the XSCID dog a valuable model for studying the role of these cytokines in B-cell development and function. Copyright (C) 2000 Elsevier Science B.V.

Original languageEnglish (US)
Pages (from-to)121-134
Number of pages14
JournalVeterinary Immunology and Immunopathology
Issue number1-2
StatePublished - Jun 30 2000


  • B-cells
  • Common gamma chain
  • Cytokine receptors
  • Cytokines
  • Dog
  • Immunodeficiency

ASJC Scopus subject areas

  • Immunology
  • veterinary(all)


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