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重型α地中海贫血中胎儿血红蛋白的异质性

Heterogeneity of fetal hemoglobin in severe alpha-thalassemia.

作者信息

Vedvick T S, Wheeler S A, Koenig H M

出版信息

Biol Neonate. 1979;36(3-4):181-4. doi: 10.1159/000241225.

Abstract

The heterogeneity of residue 136 of the gamma-chain of human hemoglobin has been determined for a patient afflicted with severe alpha-thalassemia. Separation of the cord blood sample into the various constituent hemoglobins A, F, FI and Bart's were done on a column packed with DEAE Sephadex. The amount of glycine or alanine at position 136 was determined for hemoglobins F, FI and Bart's. The ratios determined for all three hemoglobins indicated that the G gamma/A gamma ratio is the same for all three fractions and is similar to that observed in normal cord blood samples.

摘要

已对一名患有严重α地中海贫血的患者的人血红蛋白γ链第136位残基的异质性进行了测定。将脐带血样本在装有二乙氨基乙基葡聚糖(DEAE Sephadex)的柱上分离成各种组成血红蛋白A、F、FI和巴特氏血红蛋白。测定了血红蛋白F、FI和巴特氏血红蛋白第136位的甘氨酸或丙氨酸含量。所有三种血红蛋白的测定比率表明,所有三个组分的Gγ/Aγ比率相同,且与正常脐带血样本中观察到的比率相似。

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