IgD/? plasmocytoma with immunoglobulin ? light-chain genes in the germ-line configuration

Hauke, G; Epplen, Jörg T.; Chluba, Johanna; Hollmann, A; Peter, Hans

doi:10.1007/bf00917157

Cited by 3 publications

(1 citation statement)

References 39 publications

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“…19 However, it is known that an IGL gene rearrangement can occasionally be present while the IGK genes are in germline configuration. 20 Until recently, allelic exclusion was generally regarded to be a safe mechanism that guarantees the expression of a single type of antigen receptor on each lymphocyte. However, during the last few years, several reports have indicated that dual receptor expression might occur in B lymphocytes as well as in T lymphocytes because of ongoing rearrangements after a functional receptor gene has been formed and expressed.…”

Section: Introductionmentioning

confidence: 99%

Ordered recombination of immunoglobulin light chain genes occurs at the IGK locus but seems less strict at theIGL locus

Burg

Tümkaya

Boerma

et al. 2001

Blood

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Regulation of allelic and isotypic exclusion of human immunoglobulin (Ig) light-chain genes was studied in 113 chronic B-cell leukemias as a "single-cell" model that allowed complete analysis of each light chain allele. Our data show that monospecific Ig light chain expression is in about 90% of cases determined by ordered recombination: Igkappa gene (IGK) rearrangements, followed by IGK deletions and Iglambda gene (IGL) rearrangements, resulting in the presence of only one functional Ig light chain rearrangement. In about 10% (10 cases), 2 functional Ig light chain rearrangements (IGK/IGL or IGL/IGL, but not IGK/IGK) were identified. This might be explained by the fact that regulation of the ordered recombination process is not fully strict, particularly when the IGL locus is involved. Unfavorable somatic mutations followed by receptor editing might have contributed to this finding. Eight of these 10 cases indeed contained somatic mutations. In cases with 2 functional Ig light chain rearrangements, both alleles were transcribed, but monospecific Ig expression was still maintained. This suggests that in these cases allelelic exclusion is not regulated at the messenger RNA level but either at the level of translation or protein stability or via preferential pairing of Ig light and Ig heavy chains. Nevertheless, ordered rearrangement processes are the main determinant for monospecific Ig light chain expression.

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Section: Introductionmentioning

confidence: 99%

Ordered recombination of immunoglobulin light chain genes occurs at the IGK locus but seems less strict at theIGL locus

Burg

Tümkaya

Boerma

et al. 2001

Blood

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Predominant Immunoglobulin Gene Rearrangements in Two Patients with Immunodeficiency: Restricted Use of V Gene Segments and Dna Hypermethylation

et al. 1989

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Unusual Sequence of Immunoglobulin L‐Chain Rearrangements in a Gamma Heavy Chain Disease Patient

et al. 1992

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Patients with gamma heavy chain disease (gamma-HCD) generally produce incomplete immunoglobulin (Ig) gamma-heavy chains (gamma-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 patient WIN we found gamma l-HCD proteins and lambda BJP in serum and urine. WIN gamma l-HCD protein does not carry the Ig Fd 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 C gamma l-hinge region. In DNA from peripheral blood lymphocytes of patient WIN we found bands representing dominant rearrangements in one of the two alleles of the IgH, Ig kappa and Ig lambda 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 a rearranged and a non-rearranged allele of each Ig locus produces gamma-HCD protein and lambda BJP. The productive lambda-gene rearrangement in this clone thus has not been preceded by abortive rearrangements in both kappa-locus alleles. Lymphocytes with an unusual sequence of IgL-chain gene activation seem to be involved in the case of gamma-HCD described here.

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IgD/? plasmocytoma with immunoglobulin ? light-chain genes in the germ-line configuration

Cited by 3 publications

References 39 publications

Ordered recombination of immunoglobulin light chain genes occurs at the IGK locus but seems less strict at theIGL locus

Ordered recombination of immunoglobulin light chain genes occurs at the IGK locus but seems less strict at theIGL locus

Predominant Immunoglobulin Gene Rearrangements in Two Patients with Immunodeficiency: Restricted Use of V Gene Segments and Dna Hypermethylation

Unusual Sequence of Immunoglobulin L‐Chain Rearrangements in a Gamma Heavy Chain Disease Patient

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