GSBS Student Publications


B cells are required for Aire-deficient mice to develop multi-organ autoinflammation: A therapeutic approach for APECED patients

UMMS Affiliation

Graduate School of Biomedical Sciences; Research Division



Document Type


Medical Subject Headings

Animals; Antibodies; Autoimmunity; B-Lymphocytes; Cell Differentiation; Humans; *Immunotherapy; Inflammation; Mice; Mice, Inbred BALB C; Mice, Knockout; Organ Specificity; Polyendocrinopathies, Autoimmune; T-Lymphocytes; Transcription Factors


Life Sciences | Medicine and Health Sciences


Autoimmune regulator (Aire)-deficient mice and humans have circulating autoantibodies against a multitude of organs and multiorgan autoinflammatory infiltrates. It is not known to what extent autoantibodies or their source, B lymphocytes, are required for disease onset or progression. We show in this research that B cells must be present for Aire-deficient mice to develop fulminant infiltrates. We found no evidence that autoantibodies were directly pathogenic; rather, B cells appeared to play a critical early role in T cell priming or expansion. A therapeutic reagent directed against B cells, Rituximab, induced remission of the autoimmune disease in Aire-deficient mice, raising the hope of applying it to human patients with autoimmune-polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED).

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Citation: Proc Natl Acad Sci U S A. 2008 Sep 2;105(35):13009-14. Epub 2008 Aug 28. Link to article on publisher's site

DOI of Published Version


Related Resources

Link to Article in PubMed

Journal Title

Proceedings of the National Academy of Sciences of the United States of America

PubMed ID