血红蛋白的生物合成安阿伯：分解代谢和反馈调节的证据

Biosynthesis of hemoglobin Ann Arbor: evidence for catabolic and feedback regulation.

作者信息

Adams J G, Winter W P, Rucknagel D L, Spencer H H

出版信息

Science. 1972 Jun 30;176(4042):1427-9. doi: 10.1126/science.176.4042.1427.

DOI:10.1126/science.176.4042.1427

Abstract

Hemoglobin Ann Arbor, in which arginine replaces leucine in position 80 of the a chain, occurs in aflected individuals in low proportion to hemoglobin A. Biosynthetic studies were perforined on reticulocytes of a patient heterozygous for this hemoglobin. These studies suggested that the low percentage of hemoglobin Ann Arbor is prinlarily due to preferential destruction of the abnormal component. The reduced concentration of alpha Ann Arbor chains was also reflected in a decreased synthesis of normal beta chains.

摘要

血红蛋白安阿伯型，其α链第80位的亮氨酸被精氨酸取代，在受影响个体中所占比例低于血红蛋白A。对一名该血红蛋白杂合子患者的网织红细胞进行了生物合成研究。这些研究表明，血红蛋白安阿伯型比例低主要是由于异常成分被优先破坏。α安阿伯型链浓度降低也反映在正常β链合成减少上。