Globin Messenger RNA in Hemoglobin H Disease

Author:

Benz Edward J.12,Swerdlow Paul S.13,Forget Bernard G.14

Affiliation:

1. Division of Hematology of the Department of Medicine, Children‘s Hospital Medical Center, the Department of Pediatrics, Harvard Medical School, Boston, Mass. 02115.

2. Peter Bent Brigham Hospital, Boston, Mass. 02115.

3. Harvard Medical School, Boston, Macc. 02115.

4. Children's Hospital Medical Center; Assistant Professor of Pediatrics, Harvard Medical School, Boston, Mass. 02115: Recipient of Research Career Development A ward, AM-70234, of the National Institutes of Health. Bethesda, Md.

Abstract

Abstract Functional messenger RNA (mRNA) for human globin synthesis was isolated from reticulocytes of each of two patients with hemoglobin H disease. The RNA was tested for its capacity to direct globin synthesis in a messenger RNA-dependent cell-free system derived from Krebs Type II mouse ascites tumor cells. In each case, hemoglobin H disease mRNA directed the synthesis of a great excess of β-globin chains relative to α-globin chains of hemoglobin A. The β/α synthetic ratios obtained in the cell-free system at saturating concentrations of mRNA were >22 and >15, respectively, for the two hemoglobin H disease mRNA preparations, whereas the β/α synthetic ratios obtained by incubation of intact reticulocytes from these same patients were 2.6 and 2.8, respectively. The β/α synthetic ratio obtained in the cell-free system did not vary when lower concentrations of hemoglobin H disease mRNA were used. A marked decrease in the amount of functional α-globin-chain mRNA relative to β-chain mRNA is therefore associated with the decreased α-chain synthesis observed in hemoglobin H disease. This decrease in α-chain-specific mRNA activity is greater than expected from the β/α synthetic ratio of intact reticulocytes in hemoglobin H disease.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3