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• W2480043776 endingPage "960" @default.
• W2480043776 startingPage "943" @default.
• W2480043776 abstract "Normal adult hemoglobin consists of four polypeptides (two alpha chains and two beta chains) and the ironcontaining prosthetic group (heme or ferroprotoporphyrin IX). In the early embryo, theta (y) and zeta (z) chains are present instead of the alpha (a) chains, and epsilon (e) chains are present instead of the beta (b) chains. After early embryogenesis, pairs of alpha chains are linked with pairs of either beta, gamma (g), or delta (d) chains to form adult hemoglobin (Hgb A = a2b2), fetal hemoglobin (Hgb F = a2g2), or hemoglobin A2 (Hgb A2 = a2d2). By term gestation, the ratio of hemoglobin F to hemoglobin A is approximately 1:1. By 1 year of age, hemoglobin F typically constitutes less than 1% of total hemoglobin. Although hemoglobin A2 is present, it accounts for less than 2.5% of total adult hemoglobin. The sequence of amino acids (141 amino acids for alpha chains and 146 for beta chains) defines the primary structure. The three-dimensional shape of each chain defines the secondary structure, and the relationship between the four chains and the heme prosthetic group defines the tertiary structure. The binding of the ligands 2,3-diphosphoglycerate (2,3-DPG) and oxygen defines the quaternary structure. The affinity of hemoglobin for oxygen is expressed as the P50 (i.e., the oxygen tension at which half of hemoglobin’s oxygen-carrying capacity is used). Increased temperature and increases in hydrogen ion [H+] and 2,3-DPG concentrations reduce the affinity of hemoglobin for oxygen, leading to an increase in the P50 and facilitating the unloading of oxygen at peripheral tissues. In comparison with purified hemoglobin A, purified hemoglobin F has a lower oxygen affinity and a greater response to changes in pH but only a minimal response to changes in 2,3-DPG concentration. The decreased interaction between hemoglobin F and intraerythrocyte 2,3-DPG accounts for the increased affinity of fetal blood for oxygen in vivo." @default.
• W2480043776 created "2016-08-23" @default.
• W2480043776 creator A5025780649 @default.
• W2480043776 date "2009-01-01" @default.
• W2480043776 modified "2023-09-24" @default.
• W2480043776 title "Hematologic and Coagulation Disorders" @default.
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