Reichman-Fried M, Hardy R R, Bosma M J
Institute for Cancer Research, Fox Chase Cancer Center, Philadelphia, PA 19111.
Proc Natl Acad Sci U S A. 1990 Apr;87(7):2730-4. doi: 10.1073/pnas.87.7.2730.
Mice homozygous for the mutation scid (scid mice) are severely immunodeficient and generally lack detectable numbers of pre-B, B, and T cells. This condition is believed to result from a defect in the mechanism responsible for rearrangement of immunoglobulin and T-cell receptor genes in developing B and T lymphocytes. To test this hypothesis and evaluate whether scid affects only the process of gene recombination, we introduced functionally rearranged immunoglobulin genes into the scid mouse genome. As scid mice appear to contain early lymphoid cells committed to the B lineage (pro-B cells), we asked whether the introduction of an IgM heavy-chain gene alone (mu-transgenic scid mice) or both IgM heavy- and kappa light-chain genes (mu kappa-transgenic scid mice) would allow further differentiation of scid pro-B cells into pre-B and B cells. We found that normal numbers of pre-B cells appeared in the bone marrow of mu-transgenic scid mice and that both pre-B and B cells appeared in the bone marrow of mu kappa-transgenic scid mice. However, in the latter case, the number of pre-B and B cells was 2- to 3-fold less than in the controls (mu kappa-transgenic scid heterozygotes) and few, if any, B cells were detectable in the peripheral lymphoid tissues. The implications of these results for the above hypothesis are discussed.
scid突变纯合子小鼠(scid小鼠)存在严重免疫缺陷,通常缺乏可检测数量的前B细胞、B细胞和T细胞。这种情况被认为是由于负责发育中的B淋巴细胞和T淋巴细胞免疫球蛋白及T细胞受体基因重排的机制存在缺陷所致。为了验证这一假设并评估scid是否仅影响基因重组过程,我们将功能重排的免疫球蛋白基因导入scid小鼠基因组。由于scid小鼠似乎含有定向于B细胞系的早期淋巴细胞(前B细胞),我们探究单独导入IgM重链基因(μ转基因scid小鼠)或同时导入IgM重链和κ轻链基因(μκ转基因scid小鼠)是否能使scid前B细胞进一步分化为前B细胞和B细胞。我们发现,μ转基因scid小鼠骨髓中出现了正常数量的前B细胞,而μκ转基因scid小鼠骨髓中同时出现了前B细胞和B细胞。然而,在后一种情况下,前B细胞和B细胞的数量比对照组(μκ转基因scid杂合子)少2至3倍,在外周淋巴组织中几乎检测不到B细胞(若有也极少)。本文讨论了这些结果对上述假设的意义。