{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,24]],"date-time":"2026-02-24T09:33:21Z","timestamp":1771925601131,"version":"3.50.1"},"reference-count":18,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2007,8,1]],"date-time":"2007-08-01T00:00:00Z","timestamp":1185926400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2007,8,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Although null mutations in Ig\u03b1 have been identified in patients with defects in B cell development, no mutations in Ig\u03b2 have been reported. We recently identified a patient with a homozygous amino acid substitution in Ig\u03b2, a glycine to serine at codon 137, adjacent to the cysteine required for the disulfide bond between Ig\u03b1 and Ig\u03b2. This patient has a small percentage of surface IgMdim B cells in the peripheral circulation (0.08% compared with 5\u201320% in healthy controls). Using expression vectors in 293T cells or Jurkat T cells, we show that the mutant Ig\u03b2 can form disulfide-linked complexes and bring the \u03bc H chain to the cell surface as part of the BCR but is inefficient at both tasks. The results show that minor changes in the ability of the Ig\u03b1\/Ig\u03b2 complex to bring the BCR to the cell surface have profound effects on B cell development.<\/jats:p>","DOI":"10.4049\/jimmunol.179.4.2055","type":"journal-article","created":{"date-parts":[[2014,4,18]],"date-time":"2014-04-18T18:56:09Z","timestamp":1397847369000},"page":"2055-2059","source":"Crossref","is-referenced-by-count":65,"title":["Cutting Edge: A Hypomorphic Mutation in Ig\u03b2 (CD79b) in a Patient with Immunodeficiency and a Leaky Defect in B Cell Development"],"prefix":"10.1093","volume":"179","author":[{"given":"A Kerry","family":"Dobbs","sequence":"first","affiliation":[{"name":"Department of Immunology, St. Jude Children\u2019s Research Hospital , Memphis, TN 38105"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tianyu","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Immunology, St. Jude Children\u2019s Research Hospital , Memphis, TN 38105"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dana","family":"Farmer","sequence":"additional","affiliation":[{"name":"Department of Immunology, St. Jude Children\u2019s Research Hospital , Memphis, TN 38105"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Leo","family":"Kager","sequence":"additional","affiliation":[{"name":"Department of Hematology\/Oncology, St. Anna Children\u2019s Hospital , Vienna ,","place":["Austria"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ornella","family":"Parolini","sequence":"additional","affiliation":[{"name":"Centro di Ricerca E. Menni, Fondazione Poliambulanza , Brescia ,","place":["Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mary Ellen","family":"Conley","sequence":"additional","affiliation":[{"name":"Department of Immunology, St. Jude Children\u2019s Research Hospital , Memphis, TN 38105"},{"name":"Department of Pediatrics, University of Tennessee , Memphis, TN 38163"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2007,8,15]]},"reference":[{"key":"2026012809200228500_R1","first-page":"19272","volume-title":"J. Biol. Chem.","year":"1996"},{"key":"2026012809200228500_R2","first-page":"343","volume-title":"Semin. Immunol.","year":"2002"},{"key":"2026012809200228500_R3","first-page":"97","volume-title":"Annu. Rev. Immunol.","year":"1992"},{"key":"2026012809200228500_R4","first-page":"411","volume-title":"Science","year":"1996"},{"key":"2026012809200228500_R5","first-page":"865","volume-title":"J. Immunol.","year":"2002"},{"key":"2026012809200228500_R6","first-page":"1115","volume-title":"J. Clin. Invest.","year":"1999"},{"key":"2026012809200228500_R7","first-page":"455","volume-title":"J. Exp. Med.","year":"2001"},{"key":"2026012809200228500_R8","first-page":"1385","volume-title":"Int. Immunol.","year":"2006"},{"key":"2026012809200228500_R9","first-page":"1751","volume-title":"Hum. Mol. Genet.","year":"1994"},{"key":"2026012809200228500_R10","first-page":"1777","volume-title":"Blood","year":"1997"},{"key":"2026012809200228500_R11","first-page":"589","volume-title":"Nat. Biotechnol.","year":"2004"},{"key":"2026012809200228500_R12","first-page":"676","volume-title":"Leukemia","year":"2004"},{"key":"2026012809200228500_R13","first-page":"1034","volume-title":"Am. J. Hum. Genet.","year":"1998"},{"key":"2026012809200228500_R14","first-page":"3070","volume-title":"J. Immunol.","year":"1985"},{"key":"2026012809200228500_R15","first-page":"75","volume-title":"Immunol. Rev.","year":"2000"},{"key":"2026012809200228500_R16","first-page":"1029","volume-title":"J. Clin. Invest.","year":"2002"},{"key":"2026012809200228500_R17","first-page":"71","volume-title":"J. Exp. Med.","year":"1998"},{"key":"2026012809200228500_R18","first-page":"1954","volume-title":"Science","year":"1999"}],"container-title":["The Journal of Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jimmunol\/article-pdf\/179\/4\/2055\/62663562\/zim01607002055.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jimmunol\/article-pdf\/179\/4\/2055\/62663562\/zim01607002055.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,1,28]],"date-time":"2026-01-28T14:20:07Z","timestamp":1769610007000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jimmunol\/article\/179\/4\/2055\/8021590"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,8]]},"references-count":18,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2007,8,15]]},"published-print":{"date-parts":[[2007,8,15]]}},"URL":"https:\/\/doi.org\/10.4049\/jimmunol.179.4.2055","relation":{},"ISSN":["0022-1767","1550-6606"],"issn-type":[{"value":"0022-1767","type":"print"},{"value":"1550-6606","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2007,8]]},"published":{"date-parts":[[2007,8]]}}}