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Individuals with X-HIGM syndrome fail to express functional CD40 ligand; consequently they cannot mount effective protective antibody responses against pathogenic bacteria. We evaluated, compared, and characterized the humoral immune response of wild type (WT) and C57-CD40L deficient (C57-CD40L −/− ) mice infected with  Citrobacter rodentium . Basal serum isotype levels were similar for IgM and IgG3 among mice, while total IgG and IgG2b concentrations were significantly lower in C57-CD40L −/−  mice compared with WT. Essentially IgG1 and IgG2c levels were detectable only in WT mice. C57-CD40L −/−  animals, orally inoculated with 2 × 10 9  CFU, presented several clinical manifestations since the second week of infection and eventually died. In contrast at this time point no clinical manifestations were observed among C57-CD40L −/−  mice infected with 1 × 10 7  CFU. Infection was subclinical in WT mice inoculated with either bacterial dose. The serum samples from infected mice (1 × 10 7  CFU), collected at day 14 after infection, had similar  C. rodentium -specific IgM titres. Although C57-CD40L −/−  animals had lower IgG and IgG2b titres than WT mice, C57-CD40L −/−  mice sera displayed complement-mediated bactericidal activity against  C. rodentium .  C. rodentium -infected C57-CD40L −/−  mice are capable of producing antibodies that are protective. C57-CD40L −/−  mouse is a useful surrogate model of X-HIGM syndrome for studying immune responses elicited against pathogens.

biomed research international

CD40 Ligand Deficient C57BL/6 Mouse Is a Potential Surrogate Model of Human X-Linked Hyper IgM (X-HIGM) Syndrome for Characterizing Immune Responses against Pathogens