title: Polyclonal hyper-immunoglobulin G1(A1) syndrome. Evidence for a dominant immunoglobulin production regulator within the human immunoglobulin heavy chain gene complex.
abstract: The hyper-IgG1(A1) syndrome entails a polyclonal selective increase of the serum levels of immunoglobulin (Ig) G1 and to a lesser extent of IgA1; this is not mediated by malignancy, infectious or autoimmune diseases or environmental agents. In three generations of a family, all the affected individuals carried an immunoglobulin heavy chain (IgH) allele distinguished by restriction fragment length polymorphism analysis; the IgH allele was not present in non-affected family members. A 32:1 chance for the linkage of this rare IgH haplotype with the hyper-IgG1(A1) syndrome in the family argues for a dominant regulator located at the human IgH locus having a selective influence on the production of IgG1 and IgA1.
authors.affiliation: Department of Immunohaematology, University Medical Center, Leiden, The Netherlands.
authors.forename: R W
authors.initials: RW
authors.lastname: Hendriks
authors.forename: L A
authors.initials: LA
authors.lastname: Sandkuyl
authors.forename: M E
authors.initials: ME
authors.lastname: Kraakman
authors.forename: G G
authors.initials: GG
authors.lastname: de Lange
authors.forename: R K
authors.initials: RK
authors.lastname: Schuurman
mesh_terms: D005260:Female; D005786:Gene Expression Regulation; D006239:Haplotypes; D006801:Humans; D006942:Hypergammaglobulinemia / Q000235:genetics*; D007070:Immunoglobulin A / Q000235:genetics*; D007074:Immunoglobulin G / Q000235:genetics*; D007143:Immunoglobulin Heavy Chains / Q000235:genetics*; D008297:Male; D010375:Pedigree
chemical_list: D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007143:Immunoglobulin Heavy Chains