Publication Date:
1997-11-05
Description:
When transgenic mice that expressed human sickle hemoglobin were mated with mice having knockout mutations of the mouse alpha- and beta-globin genes, animals were produced that synthesized only human hemoglobin in adult red blood cells. Similar to many human patients with sickle cell disease, the mice developed a severe hemolytic anemia and extensive organ pathology. Numerous sickled erythrocytes were observed in peripheral blood. Although chronically anemic, most animals survived for 2 to 9 months and were fertile. Drug and genetic therapies can now be tested in this mouse model of sickle cell disease.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Ryan, T M -- Ciavatta, D J -- Townes, T M -- CA13148/CA/NCI NIH HHS/ -- New York, N.Y. -- Science. 1997 Oct 31;278(5339):873-6.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Department of Biochemistry and Molecular Genetics, Schools of Medicine and Dentistry, University of Alabama at Birmingham, Birmingham, AL 35294, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/9346487" target="_blank"〉PubMed〈/a〉
Keywords:
*Anemia, Sickle Cell/blood/genetics/pathology
;
Animals
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Chromatography, High Pressure Liquid
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Crosses, Genetic
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*Disease Models, Animal
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Erythrocytes/pathology
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Globins/genetics
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Hemoglobin, Sickle/genetics
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Hemoglobins/genetics
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Humans
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Mice
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Mice, Inbred C57BL
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Mice, Knockout
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Mice, Transgenic
Print ISSN:
0036-8075
Electronic ISSN:
1095-9203
Topics:
Biology
,
Chemistry and Pharmacology
,
Computer Science
,
Medicine
,
Natural Sciences in General
,
Physics
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