{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T14:57:12Z","timestamp":1761058632896},"reference-count":26,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2005,12,14]],"date-time":"2005-12-14T00:00:00Z","timestamp":1134518400000},"content-version":"vor","delay-in-days":5492,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur J Immunol"],"published-print":{"date-parts":[[1990,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>X\u2010linked immunodeficiency with hyperimmunoglobulinemia M (XHM) reflects an impairment of the immunoglobulin (Ig) heavy (H) chain class switch of B lymphocytes from IgM to IgG and IgA.<\/jats:p><jats:p>XHM is recessive; female carriers manifest normal IgG and IgA production. Due to random X chromosome inactivation in all somatic cells of females, about half of the lymphocytes of XHM carriers are not able to express an intact XHM gene. An intrinsic defect of the Ig H chain class switch mechanism in XHM B lymphocytes would thus lead to a skewed X chromosome inactivation pattern in the IgG\u2010 and IgA\u2010expressing B lymphocytes of female carriers.<\/jats:p><jats:p>IgM\u2010, IgG\u2010 and IgA\u2010expressing B lymphoblastoid cells (BLC) were established by Epstein\u2010Barr virus transformation of peripheral blood mononuclear cells of two female XHM carriers. In an analysis of differential methylation of the polymorphic DXS255 loci, random X chromosome inactivation patterns were found in populations of T lymphocytes, in IgM\u2010expressing B lymphocytes and in IgG\u2010 or IgA\u2010expressing B lymphocytes. The heterogeneity of Ig H chain rearrangements and the Ig light chain usage in the IgA\u2010 or IgG\u2010expressing BLC clones that had inactivated the X chromosome which carries the intact XHM gene and in BLC clones with the homologous X chromosome inactivated were similar.<\/jats:p><jats:p>The results indicated that the intrinsic Ig H chain class switch mechanism in XHM B lymphocytes is fully intact. We conclude that the XHM gene encodes a class switch inducer that is transferred to B lymphocytes.<\/jats:p>","DOI":"10.1002\/eji.1830201212","type":"journal-article","created":{"date-parts":[[2007,3,1]],"date-time":"2007-03-01T11:54:29Z","timestamp":1172750069000},"page":"2603-2608","source":"Crossref","is-referenced-by-count":37,"title":["Evidence that in X\u2010linked immunodeficiency with hyperimmunoglobulinemia M the intrinsic immunoglobulin heavy chain class switch mechanism is intact"],"prefix":"10.1002","volume":"20","author":[{"given":"Rudolf W.","family":"Hendriks","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Margriet E. M.","family":"Kraakman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ian W.","family":"Craig","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Teresa","family":"Espanol","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ruud K. 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