Keller G M, Phillips R A
Exp Hematol. 1984 Dec;12(11):822-4.
Although phenotypically similar, the cellular defects in congenitally anemic mice of genotype W/Wv and S1/S1d are quite different. W/Wv mice have defective hemopoietic stem cells; in contrast, S1/S1d mice have normal stem cells, but their hemopoietic microenvironment cannot support normal differentiation of the stem cells. We also observed defective hemopoiesis as measured by granulocyte-macrophage colony-forming units (GM-CFU) in long-term bone marrow cultures (LTBMC) established with marrow from these mutants, but in contrast to an earlier report we obtained long-term maintenance of hemopoiesis (up to 20 weeks) albeit at a lower level than the control (28% of control for S1/S1d and 23% for W/Wv). These levels probably reflect more accurately the in vivo effects of the mutations than the severe defect reported previously. However, this level of hemopoiesis and the high variability observed in replicate flasks in LTBMC make it difficult to study these mutants in tissue culture.
尽管在表型上相似，但基因型为W/Wv和S1/S1d的先天性贫血小鼠的细胞缺陷却大不相同。W/Wv小鼠的造血干细胞存在缺陷；相反，S1/S1d小鼠的干细胞正常，但其造血微环境无法支持干细胞的正常分化。在用这些突变体的骨髓建立的长期骨髓培养（LTBMC）中，我们也观察到以粒细胞-巨噬细胞集落形成单位（GM-CFU）衡量的造血缺陷，但与早期报告不同的是，我们实现了造血的长期维持（长达20周），尽管其水平低于对照组（S1/S1d为对照组的28%，W/Wv为23%）。这些水平可能比先前报道的严重缺陷更准确地反映了体内突变的影响。然而，这种造血水平以及在LTBMC的重复培养瓶中观察到的高变异性使得在组织培养中研究这些突变体变得困难。
