Aiolos regulates B cell activation and maturation to effector state.
Aiolos encodes a zinc finger DNA-binding protein that is highly expressed in mature B cells and is homologous to Ikaros. In the periphery of mice homozygous for an Aiolos-null mutation, B cells exhibit an activated cell surface phenotype and undergo augmented antigen receptor (BCR)-mediated in vitro proliferative responses, even at limiting amounts of stimulant. In vivo, T cell-dependent B cell responses, including the formation of germinal centers and elevated serum IgG and IgE, are detected in Aiolos-deficient mice in the absence of immunization. Auto-antibodies and development of B cell lymphomas are frequently seen among aging Aiolos mutants. In sharp contrast to conventional B cells, B cells of the peritoneum, of the marginal zone, and the recirculating bone marrow population are greatly reduced.
Wang
J H
JH
Cutaneous Biology Research Center, Massachusetts General Hospital, Harvard Medical School, Charlestown 02129, USA.
Avitahl
N
N
Cariappa
A
A
Friedrich
C
C
Ikeda
T
T
Renold
A
A
Andrikopoulos
K
K
Liang
L
L
Pillai
S
S
Morgan
B A
BA
Georgopoulos
K
K
D000818:Animals; D001323:Autoantibodies / Q000097:blood; D001402:B-Lymphocytes / Q000166:cytology / Q000276:immunology*; D001483:Base Sequence; D002454:Cell Differentiation; D016207:Cytokines / Q000096:biosynthesis; D017931:DNA Primers / Q000235:genetics; D004268:DNA-Binding Proteins; D006412:Hematopoietic Stem Cells / Q000166:cytology / Q000276:immunology; D051740:Ikaros Transcription Factor; D007136:Immunoglobulins / Q000097:blood; D066298:In Vitro Techniques; D008213:Lymphocyte Activation; D016393:Lymphoma, B-Cell / Q000209:etiology / Q000473:pathology; D046911:Macromolecular Substances; D051379:Mice; D018345:Mice, Knockout; D010641:Phenotype; D013601:T-Lymphocytes / Q000276:immunology; D015534:Trans-Activators / Q000737:chemistry / Q000235:genetics / Q000276:immunology*; D014157:Transcription Factors / Q000737:chemistry / Q000276:immunology; D016335:Zinc Fingers / Q000235:genetics / Q000276:immunology
D001323:Autoantibodies; D016207:Cytokines; D017931:DNA Primers; D004268:DNA-Binding Proteins; C491396:Ikzf3 protein, mouse; D007136:Immunoglobulins; D046911:Macromolecular Substances; D015534:Trans-Activators; D014157:Transcription Factors; C497238:Zfpn1a1 protein, mouse; D051740:Ikaros Transcription Factor