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Two of the genes involved in these diseases have been isolated, namely the genes for X\u2010linked chronic granulomatous disease and properdin deficiency, and substantial progress has now been made in identifying the genes which are defective in the other five diseases, Wiskott\u2010Aldrich syndrome, X\u2010linked severe combined immunodeficiency, X\u2010linked agammaglobulinaemia, X\u2010linked hyper\u2010IgM and X\u2010linked lymphoproliferative syndrome. We review here the nature of the diseases, progress made in identifying and isolating the genes involved and the prospects for improved prenatal detection, carrier status determination and treatment of these life\u2010threatening conditions.<\/jats:p>","DOI":"10.1007\/bf01799623","type":"journal-article","created":{"date-parts":[[2005,6,21]],"date-time":"2005-06-21T17:25:03Z","timestamp":1119374703000},"page":"674-682","source":"Crossref","is-referenced-by-count":7,"title":["The molecular basis of X\u2010linked immunodeficiency disease"],"prefix":"10.1002","volume":"15","author":[{"given":"C.","family":"Kinnon","sequence":"first","affiliation":[{"name":"Molecular Immunology Unit, Institute of Child Health University of London  30 Guilford Street London WC1N 1EH UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R.","family":"Levinsky","sequence":"additional","affiliation":[{"name":"Molecular Immunology Unit, Institute of Child Health University of London  30 Guilford Street London WC1N 1EH UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1992,7]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0090-1229(90)90133-B"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.84.21.7576"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V76.3.602.602"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(88)91537-1"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00210662"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.1991.tb05579.x"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/0888-7543(88)90092-4"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/0888-7543(89)90085-2"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/0888-7543(89)90315-7"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/0888-7543(90)90489-H"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00210664"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/eji.1830201212"},{"key":"e_1_2_1_14_1","first-page":"266","volume-title":"Progress in Immune Deficiency III","author":"Hendriks RW","year":"1991"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/0888-7543(90)90562-9"},{"key":"e_1_2_1_16_1","first-page":"595","article-title":"Phorbol\u2010myristate acetate stimulated NBT test; 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