Aiolos encodes a zinc finger DNA-binding protein that is highly expres
sed in mature B cells and is homologous to Ikaros. In the periphery of
mice homozygous for an Aiolos-null mutation, B cells exhibit an activ
ated cell surface phenotype and undergo augmented antigen receptor (BC
R)-mediated in vitro proliferative responses, even at limiting amounts
of stimulant. In vivo, T cell-dependent B cell responses, including t
he formation of germinal centers and elevated serum IgG and IgE, are d
etected in Aiolos-deficient mice in the absence of immunization. Auto-
antibodies and development of B cell lymphomas are frequently seen amo
ng aging Aiolos mutants. In sharp contrast to conventional B cells, B
cells of the peritoneum, of the marginal zone, and the recirculating b
one marrow population are greatly reduced.