Three mouse models of human thalassemia

Journal Article · Sat Aug 01 00:00:00 EDT 1981 · Proc. Natl. Acad. Sci. U.S.A.; (United States)

DOI:https://doi.org/10.1073/pnas.78.8.5056· OSTI ID:5952744

Martinell, J ^[1]; Whitney, J B; Popp, R A; Russell, L B; Anderson, W F

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1. National Institutes of Health, Betheseda, MD

Three types of mice with globin gene mutations, called 352HB, 27HB, and Hba/sup th-J/, appear to be true animal models of human thalassemia. Expression of the ..cap alpha..-globin genes in three stocks of mice, each one heterozygous for one of the ..cap alpha..-globin mutations, was examined at the polypeptide, RNA, and DNA levels. ..cap alpha..-globin polypeptide chains, relative to ..gamma..-globin chains in heterozygous thalassemic mice, are present at approximately 80% of normal. The ratios of ..cap alpha..-globin to ..gamma..-globin RNA sequences are also 75 to 80% normal, exactly reflecting the ..cap alpha..-globin to ..gamma..-globin chain ratios. In the case of mutant 352HB, at least one ..cap alpha..-globin gene is deleted. Thalassemic mouse erythroid cells appear to compensate partially for the loss of half of their ..cap alpha..-globin genes.

• OSTI ID: 5952744
• Journal Information: Proc. Natl. Acad. Sci. U.S.A.; (United States), Journal Name: Proc. Natl. Acad. Sci. U.S.A.; (United States) Vol. 78:8; ISSN PNASA
• Country of Publication: United States
• Language: English

Related Subjects

550400* -- Genetics
59 BASIC BIOLOGICAL SCIENCES
ANEMIAS
ANIMALS
BIOCHEMISTRY
BIOLOGICAL MODELS
BIOLOGY
CHEMISTRY
DISEASES
DNA
GENES
GENETICS
GLOBIN
HEMIC DISEASES
HYBRIDIZATION
MAMMALS
MICE
MUTAGENESIS
MUTATIONS
NATIONAL ORGANIZATIONS
NUCLEIC ACIDS
ORGANIC COMPOUNDS
PROTEINS
RESEARCH PROGRAMS
RNA
RODENTS
SYMPTOMS
THALASSEMIA
US DOE
US ORGANIZATIONS
VERTEBRATES