Hb蒙蒂菲奥里(126 (H9) Asp - - >酪氨酸)。高氧亲和力和协调二级c端中断的损失。
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Wajcman H, kist J, Galacteros F,任务,林MJ, Vidugiris GJ,赫希再保险,弗里德曼JM,内格尔RL
Hb蒙蒂菲奥里(126 (H9) Asp - - >酪氨酸)。高氧亲和力和协调二级c端中断的损失。
生物化学杂志。1996年9月20日;271 (38):22990 - 8。
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8798486 (在PubMed]
- 文摘
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Hb蒙蒂菲奥里被发现,杂合的状态,在波多黎各的女性总Hb水平轻微升高。结构分析表明,Asp-alpha126被酪氨酸所取代。Hb蒙蒂菲奥里迁移靠近住宅(pH值8.6)和hemolysate的占20.3%。氧气结合红细胞透露P50下降40% (pH值7.4)和低n值为1.6(正常:2.6)。损耗的红细胞2,3-DPG改变不了结果。剥夺了Hb蒙蒂菲奥里在P50 pH值降低7.2显示一个8倍(0.6和4.6毫米汞柱),非常低的协调控制(n = 1.2和2.9)。异位的效应器,2、3-diphosphoglycerate和肌醇hexaphosphate正常效果,此外,他们增加了协调。氯离子效应和玻尔效应适度减少。苯扎贝特导数(L345),已知alpha126结合,增加了P50 HbA的9倍,但只有1。Hb蒙蒂菲奥里的5倍。 Combining these functional studies with intrinsic fluorescence and Resonance Raman spectroscopy, we interpret the very low n value and the high oxygen affinity for Hb Montefiore as a result of both a destabilized T state that switches to R upon ligand binding and a deoxy T state that binds ligands with higher affinity than that of deoxy HbA. Hb Montefiore still binds ligands cooperatively, but the difference in ligand binding properties of the two quaternary states has been drastically reduced.