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Unusual Sequence of Immunoglobulin L‐Chain Rearrangements in a Gamma Heavy Chain Disease Patient
Author(s) -
HAUKE G.,
SCHILTZ E.,
BROSS K. J.,
HOLLMANN A.,
PETER H. H.,
KRAWINKEL U.
Publication year - 1992
Publication title -
scandinavian journal of immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.934
H-Index - 88
eISSN - 1365-3083
pISSN - 0300-9475
DOI - 10.1111/j.1365-3083.1992.tb02961.x
Subject(s) - immunoglobulin heavy chain , locus (genetics) , allele , gene , antibody , immunoglobulin light chain , gene rearrangement , biology , microbiology and biotechnology , genetics , clone (java method)
Patients with gamma heavy chain disease (γ‐HCD) generally produce incomplete immunoglobulin (Ig) γ‐heavy chains (yγ‐HCD protein) which cannot associate with light chains (IgL). In most patients Bence Jones proteins (BJP) are not observed. However, in the 61‐year‐old patienl WIN we found γl‐HCD proteins and λ BJP in serum and urine. WIN ‐γl‐HCD protein does not carry the Ig F d region, has a molecular weight of 33.5 kDa, and the seven N‐terminal amino acid residues are not translated from any of the known immunoglobulin heavy chain (IgH) gene sequences. These residues are followed by the Cγl‐hinge region. In DNA from peripheral blood lymphocytes of patient WIN we found bands representing dominant rearrangements in one ofthe two alleles of the IgH, IgK and Igλ locus. Taken together, the data from protein and DNA analysis strongly suggest, albeit do not formally prove, that one dominant B‐cell clone which carries it rearranged and a non‐rearranged allele of each Ig locus produces γ‐HCD protein and λ BJP. The productive λ‐gene rearrangement in this clone thus has not been preceded by abortive rearrangements in both γ‐locus alleles. Lymphocytes with an unusual sequence of IgL‐chain gene activation seem to be involved in the case of γ‐HCD described here.