The wasted mutant mouse. I. An animal model of secretory IgA deficiency with normal serum IgA.
The wasted (wst) mutation was recently described as a spontaneous, recessive mutation leading to pathologic changes affecting both the neurologic and the immune systems of wst/wst homozygotes, which presented symptoms analogous to those observed in patients with ataxia-telangiectasia (A.T.). We studied the IgA system of wst/wst mutants and their normal littermates to determine whether IgA deficiency commonly found in A.T. patients was also affecting these mutants. Interestingly, although IgA plasma cells were totally absent from their entire (small and large) intestine, their serum contained a normal level of IgA with a normal ratio of monomeric vs polymeric IgA. The absence of gut IgA plasma cells was not due to malnutrition and was not compensated by the appearance of cells secreting any other isotypes. Studies at the precursor cell level showed the absence of IgA-specific B cell precursors in the Peyer's patches, whereas sIgA B cells and IgA plasma cells were found in normal numbers in the spleen of wasted mice. These data suggest that secretory and serum IgA may comprise distinct systems and that the wasted mutant mouse is a potential model for the study of the physiology and regulation of IgA production.
D000818:Animals; D001402:B-Lymphocytes / Q000145:classification; D002452:Cell Count; D004195:Disease Models, Animal; D004406:Dysgammaglobulinemia / Q000235:genetics / Q000276:immunology*; D005260:Female; D006720:Homozygote; D007070:Immunoglobulin A / Q000378:metabolism; D007071:Immunoglobulin A, Secretory / Q000172:deficiency*; D007413:Intestinal Mucosa / Q000166:cytology; D008221:Lymphoid Tissue / Q000166:cytology; D051379:Mice; D008809:Mice, Inbred C3H; D008810:Mice, Inbred C57BL; D008817:Mice, Mutant Strains / Q000276:immunology*; D009748:Nutrition Disorders / Q000276:immunology; D010950:Plasma Cells / Q000378:metabolism; D013234:Stem Cells / Q000145:classification; D013601:T-Lymphocytes / Q000145:classification
D007070:Immunoglobulin A; D007071:Immunoglobulin A, Secretory