Oxygen association-dissociation and stability analysis on mouse hemoglobins with mutant alpha- and beta-globins.
Oxygen association-dissociation and stability analysis on mouse hemoglobins with mutant alpha- and beta-globins.
复制标题
具有突变 α 和 β 珠蛋白的小鼠血红蛋白的氧结合-解离和稳定性分析。
DOI：
10.1093/genetics/132.2.545
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发表时间：
1992
期刊：
影响因子：
3.3
通讯作者：
Popp,RA
中科院分区：
文献类型：
--
作者：
D'Surney,SJ;Popp,RA
对α-珠蛋白(α-89，His to Leu)和β-珠蛋白(β59，Lys to Ile)中氨基酸取代的小鼠血红蛋白进行了氧缔合-解离和血红蛋白稳定性分析。突变的α-珠蛋白在Hbag2单倍型中被命名为5M链，具有高的氧亲和力并且稳定。Hbbs2单倍型的变异型β-珠蛋白(βS2)也有较高的氧亲和力，此外在19%异丙醇中也是中等不稳定的。来自预期的9种(Hbag2/Hbag2；Hbbs/Hbbs x Hbaa/Hbaa；Hbbs2/Hbbs2)F2基因型的血红蛋白可以分为五类P50值，其特征是严格的可加性和对突变的珠蛋白基因剂量的依赖；从生理上讲，这两种珠蛋白变体对氧亲和力的影响是不可区分的。正常小鼠(Hbaa/Hbaa；Hbbs/Hbbs)的P50=40 mm Hg，Hbag2/Hbag2；Hbbs2/Hbbs2 F2小鼠的P50=25 mm Hg(人P50=26 mm Hg)。Hbag2/Hbag2；Hbbs/Hbbs，Hbaa/Hbaa；Hbbs2/Hbbs2和Hbag2/Hbag2；Hbbs2/Hbbs2小鼠的血液学指标正常，但Hbag2/Hbag2；Hbbs/Hbbs和Hbag2/Hbag2；Hbbs2/Hbbs2小鼠的红细胞计数略有增加，Hbag2/Hbag2；Hbbs2/Hbbs2小鼠的平均红细胞体积和平均红细胞血红蛋白明显降低。
Oxygen association-dissociation and hemoglobin stability analysis were performed on mouse hemoglobins with amino acid substitutions in an alpha-globin (alpha 89, His to Leu) and a beta-globin (beta 59, Lys to Ile). The variant alpha-globin, designated chain 5m in the Hbag2 haplotype, had an high oxygen affinity and was stable. The variant beta-globin, (beta s2) of the Hbbs2 haplotype, also had an elevated oxygen affinity and in addition was moderately unstable in 19% isopropanol. Hemoglobins from the expected nine (Hbag2/Hbag2;Hbbs/Hbbs x Hbaa/Hbaa;Hbbs2/Hbbs2) F2 genotypes can be grouped into five classes of P50 values characterized by strict additivity and dependency on mutant globin gene dosage; physiologically, both globin variants gave indistinguishable effects on oxygen affinity. The hemoglobin of normal mice (Hbaa/Hbaa;Hbbs/Hbbs) had a P50 = 40 mm Hg and the hemoglobin of Hbag2/Hbag2;Hbbs2/Hbbs2 F2 mice had a P50 = 25 mm Hg (human P50 = 26 mm Hg). Peripheral blood from Hbag2/Hbag2;Hbbs/Hbbs, Hbaa/Hbaa;Hbbs2/Hbbs2 and Hbag2/Hbag2;Hbbs2/Hbbs2 mice exhibited normal hematological values except for a slightly higher hematocrit for Hbag2/Hbag2;Hbbs/Hbbs and Hbag2/Hbag2;Hbbs2/Hbbs2 mice, slightly elevated red cell counts for mice of the three mutant genotypes, and significantly lower values for the mean corpuscular volume and mean corpuscular hemoglobin for Hbag2/Hbag2;Hbbs2/Hbbs2 mice.