Hemoglobin subunit alpha

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.

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.

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.{{cite web | title = Entrez Gene: HBA1 hemoglobin, alpha 1 | url = https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=3039 }}

Hemoglobin subunit alpha has been shown to interact with hemoglobin subunit beta (HBB).{{cite journal |vauthors=Stelzl U, Worm U, Lalowski M, Haenig C, Brembeck FH, Goehler H, Stroedicke M, Zenkner M, Schoenherr A, Koeppen S, Timm J, Mintzlaff S, Abraham C, Bock N, Kietzmann S, Goedde A, Toksöz E, Droege A, Krobitsch S, Korn B, Birchmeier W, Lehrach H, Wanker EE | title = A human protein-protein interaction network: a resource for annotating the proteome | journal = Cell | volume = 122 | issue = 6 | pages = 957–68 |date=September 2005 | pmid = 16169070 | doi = 10.1016/j.cell.2005.08.029 | hdl = 11858/00-001M-0000-0010-8592-0 | s2cid = 8235923 | hdl-access = free }}{{cite journal | author = Shaanan B | title = Structure of human oxyhaemoglobin at 2.1 A resolution | journal = J. Mol. Biol. | volume = 171 | issue = 1 | pages = 31–59 |date=November 1983 | pmid = 6644819 | doi = 10.1016/S0022-2836(83)80313-1 }}

• Hemoglobin subunit beta
• Human β-globin locus

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{{refend}}

• [https://www.ncbi.nlm.nih.gov/books/NBK1435/ GeneReviews/NCBI/NIH/UW entry on Alpha-Thalassemia]
• [https://www.ncbi.nlm.nih.gov/omim/141800,141850,142310,604131,141800,141850,142310,604131 OMIM entries on Alpha-Thalassemia]
• {{PDBe-KB2|P69905|Hemoglobin subunit alpha}}

{{PDB Gallery|geneid=3039}}

{{Hemeproteins}}

Category:Hemoglobins

{{DEFAULTSORT:Hemoglobin, alpha 1}}