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Blood, 15 September 2005, Vol. 106, No. 6, pp. 2069-2075.
Prepublished online as a Blood First Edition Paper on June 7, 2005; DOI 10.1182/blood-2004-07-2731.
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Submitted July 16, 2004
Accepted May 22, 2005
Impaired Ig class switch in mice deficient for the X-linked lymphoproliferative disease gene sap
Umaima Al-Alem, Cuiling Li, Nathalie Forey, Francis Relouzat, Marie-Claude Fondaneche, Sean V Tavtigian, Zhao-Qi Wang, Sylvain Latour, and Luo Yin*
International Agency for Research on Cancer, Lyon, France; Division of Pulmonary, Allergy, and Critical Care Medicine, Columbia University, New York, USA
International Agency for Research on Cancer, Lyon, France
Unite INSERM 429, Hopital Necker-Enfants Malades, Paris, France
* Corresponding author; email: yinluo2005{at}hotmail.com.
X-linked lymphoproliferative disease is characterized by abnormal immune responses to Epstein-Barr virus attributed to inactivating mutations of the SAP gene. Previous studies showed IgE deficiency and low serum IgG levels in sap-deficient mice before and after viral infections, which are associated with impaired CD4+ T helper function. In the present work, we find that SAP is expressed in B cells and this expression is down-regulated after stimulation with LPS and IL-4. We demonstrate that B cells from sap-deficient mice exhibit reduced IgG and IgA production in vitro. This impairment correlates with decreased circular transcript levels of I , I 2a, I2b, and I3 after stimulation, which indicate a defective Ig switch recombination in sap-deficient B cells. While XLP is believed to cause defects in T, NKT, and NK cells, our results indicate that Ig class switching in B cells is also affected.
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