Chen J, Trounstine M, Kurahara C, Young F, Kuo C C, Xu Y, Loring J F, Alt F W, Huszar D
Howard Hughes Medical Institute, Children's Hospital, Boston, MA.
EMBO J. 1993 Mar;12(3):821-30. doi: 10.1002/j.1460-2075.1993.tb05722.x.
We have generated mice that lack the ability to produce immunoglobulin (Ig) kappa light chains by targeted deletion of J kappa and C kappa gene segments and the intervening sequences in mouse embryonic stem cells. In wild type mice, approximately 95% of B cells express kappa light chains and only approximately 5% express lambda light chains. Mice heterozygous for the J kappa C kappa deletion have approximately 2-fold more lambda+ B cells than wild-type littermates. Compared with normal mice, homozygous mutants for the J kappa C kappa deletion have about half the number of B cells in both the newly generated and the peripheral B cell compartments, and all of these B cells express lambda light chains in their Ig. Therefore, homozygous mutant mice appear to produce lambda-expressing cells at nearly 10 times the rate observed in normal mice. These findings demonstrate that kappa gene assembly and/or expression is not a prerequisite for lambda gene assembly and expression. Furthermore, there is no detectable rearrangement of 3' kappa RS sequences in lambda+ B cells of the homozygous mutant mice, thus rearrangements of these sequences, per se, is not required for lambda light chain gene assembly. We discuss these findings in the context of their implications for the control of Ig light chain gene rearrangement and potential applications of the mutant animals.
我们通过在小鼠胚胎干细胞中靶向缺失Jκ和Cκ基因片段及中间序列,培育出了缺乏产生免疫球蛋白(Ig)κ轻链能力的小鼠。在野生型小鼠中,约95%的B细胞表达κ轻链,只有约5%表达λ轻链。JκCκ缺失的杂合小鼠的λ+B细胞比野生型同窝小鼠多约2倍。与正常小鼠相比,JκCκ缺失的纯合突变体在新生和外周B细胞区室中的B细胞数量约为正常小鼠的一半,并且所有这些B细胞在其Ig中都表达λ轻链。因此,纯合突变体小鼠产生表达λ的细胞的速率似乎是正常小鼠中观察到的近10倍。这些发现表明,κ基因组装和/或表达不是λ基因组装和表达的先决条件。此外,在纯合突变体小鼠的λ+B细胞中未检测到3'κRS序列的重排,因此这些序列本身的重排对于λ轻链基因组装不是必需的。我们将在这些发现对Ig轻链基因重排控制的影响以及突变动物潜在应用的背景下讨论这些发现。