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

van der Burg, Mirjam; Tü; mkaya, Talip; Boerma, Marjan; de Bruin-Versteeg, Sandra; Langerak, Anton W.; van Dongen, Jacques J. M.

doi:10.1182/blood.v97.4.1001

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

Blood, v.97 no.4, 2001년, pp.1001 - 1008

van der Burg, Mirjam (From the Department of Immunology, Erasmus University Rotterdam) , Tümkaya, Talip (From the Department of Immunology, Erasmus University Rotterdam) , Boerma, Marjan (From the Department of Immunology, Erasmus University Rotterdam) , de Bruin-Versteeg, Sandra (From the Department of Immunology, Erasmus University Rotterdam) , Langerak, Anton W. (From the Department of Immunology, Erasmus University Rotterdam) , van Dongen, Jacques J. M. (From the Department of Immunology, Erasmus University Rotterdam)

Abstract ▼ AI-Helper

AbstractRegulation 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: Igκ gene (IGK) rearrangements, followed byIGK deletions and Igλ 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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표제어: PCR

동의어: Packet Collision Rate

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

Abstract ▼ AI-Helper

참고문헌 (40)

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