Krishnamoorthy R, Wajcman H, Labie D, Backef C H, Tron P
Biomedicine. 1976 Jun;25(4):151-4.
In Hemoglobins M, as the result of a mutation theheme iron of the abnormal chain is atabilized in an oxidized form unable to carry oxygen. In this paper, we discuss the case of hemoglobin M Boston characterizing this abnormal hemoglobin as valency hybrid by spectral studies and utilising this mutant as a marker, in isoelectricfocusing, we bring the arguments that the two intermediary bands of oxidation correspond to alpha2+beta2+2 and alpha2+2beta2+3 We also indicate that the abnormal spectrum of Hb M interferes with the estimation of methemoglobin, giving erroneous values. We have shown that isoelectric focusing and subsequent scanning give a definite idea about the amount of abnormal chain present.
在血红蛋白M中,由于突变,异常链的血红素铁以氧化形式稳定存在,无法携带氧气。在本文中,我们讨论了血红蛋白M波士顿型的病例,通过光谱研究将这种异常血红蛋白表征为价态杂合子,并利用这种突变体作为标记物。在等电聚焦中,我们提出论据,认为两个氧化中间带分别对应于α2+β2+2和α2+2β2+3。我们还指出,血红蛋白M的异常光谱会干扰高铁血红蛋白的估算,给出错误的值。我们已经表明,等电聚焦及随后的扫描能明确了解异常链的存在量。