Hemoglobin subunit alpha
Human hemoglobin protein From Wikipedia, the free encyclopedia
Hemoglobin subunit alpha, Hemoglobin, alpha 1,[5] is a hemoglobin protein that in humans is encoded by the HBA1 gene.[6]
Gene
The human alpha globin gene cluster located on chromosome 16 spans about 30 kb and includes seven loci: 5'- zeta - pseudozeta - mu - pseudoalpha-1 - alpha-2 - alpha-1 - theta - 3'. The alpha-2 (HBA2) and alpha-1 (HBA1; this gene) coding sequences are identical. These genes differ slightly over the 5' untranslated regions and the introns, but they differ significantly over the 3' untranslated regions.[6]
Protein
Two alpha chains plus two beta chains constitute HbA, which in normal adult life accounts for about 97% of the total hemoglobin; alpha chains combine with delta chains to constitute HbA-2, which with fetal hemoglobin (HbF), composed of alpha and gamma chains, make up the remaining 3% of adult hemoglobin.[6]
Clinical significance
Alpha thalassemias result from deletions of each of the alpha genes as well as deletions of both HBA2 and HBA1; some nondeletion alpha thalassemias have also been reported.[6]
Interactions
Hemoglobin subunit alpha has been shown to interact with hemoglobin subunit beta (HBB).[7][8]
See also
References
Further reading
External links
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