PMID: 2498199May 1, 1989Paper

Polyclonal hyper-immunoglobulin G1(A1) syndrome. Evidence for a dominant immunoglobulin production regulator within the human immunoglobulin heavy chain gene complex

Human Genetics
R W HendriksR K Schuurman

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.

References

Oct 1, 1986·Proceedings of the National Academy of Sciences of the United States of America·E S Lander, D Botstein
Mar 25, 1988·Nucleic Acids Research·R W HendriksR K Schuurman
Feb 1, 1986·Immunological Reviews·P D GregorS L Morrison
Jan 1, 1983·Proceedings of the National Academy of Sciences of the United States of America·N MigoneL L Cavalli-Sforza
Nov 1, 1983·Proceedings of the National Academy of Sciences of the United States of America·N T Bech-HansenD W Cox
Jan 1, 1983·Annual Review of Immunology·R Wall, M Kuehl
Nov 1, 1980·Proceedings of the National Academy of Sciences of the United States of America·J V RavetchP Leder

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