Expression of the globin genes and hematopoiesis in beta-thalassemic mice.
Expression of the globin genes and hematopoiesis in beta-thalassemic mice.
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β-地中海贫血小鼠中球蛋白基因的表达和造血作用。
DOI：
10.1007/978-1-4613-0623-8_17
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发表时间：
1989
影响因子：
--
通讯作者：
Popp,DM
中科院分区：
文献类型：
--
作者：
Popp,RA;Bolch,SL;Shinpock,SG;Popp,DM
缺失β-主要珠蛋白基因的纯合子小鼠表现出人类β-地中海贫血的临床症状，是研究珠蛋白基因表达调节、造血紊乱以及治疗β-地中海贫血患者的潜在方法的良好实验动物。纯合子β-地中海贫血小鼠患有小细胞性贫血，他们的红细胞表现为红细胞不等、多核细胞增多和寿命缩短，由于红细胞生成增加，几个组织中会出现铁超载2，3。β-地中海贫血1，2突变杂合子小鼠临床正常。
Mice homozygous for a deletion of the beta-dmajor globin gene exhibit clinical symptoms of human beta-thalassemia and are good experimental animals for investigating the regulation of globin gene expression, perturbation of hematopoiesis, and potential methods for treating patients with beta-thalassemia. Homozygous beta-thalassemic mice have a microcytic anemia, their red blood cells display anisocytosis, poikilocytosis and a shortened life span, and iron overloading occurs in several tissues in response to increased erythropoiesis2,3. Mice heterozygous for the beta-thalassemia1,2mutation are clinically normal.