Synthesis of Hemoglobin Abraham Lincoln (ß 32 leu →pro)

• Published in: Blood Vol. 43; no. 5; pp. 657 - 664
• Main Authors: Honig, George R., Mason, R. George, Vida, Loyda N., Shamsuddin, Mir
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-05-1974
• Subjects: Anemia, Hemolytic - blood; Arginine - metabolism; Carbon Radioisotopes; Globins - analysis; Hemoglobins, Abnormal - biosynthesis; Humans; Leucine - metabolism; Peptides - analysis; Peptides - isolation & purification; Proteins - analysis; Reticulocytes - metabolism; Trypsin - pharmacology
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood.V43.5.657.657

Globin chain synthesis was studied in vitro with reticulocytes from a patient heterozygous for Hb Abraham Lincoln, an unstable beta chain variant. Synthesis of α-chains by the reticulocytes exceeded total β-chain synthesis, and a substantial fraction (about 16%) of radioactivity incorporated into globin was recovered from the cells as uncombined α subunits. In a time-course study, the ratio of α: β-chain specific activity was found to increase progressively in a nearly linear manner, suggesting that a fraction of newly synthesized β-chains had undergone rapid destruction. The specific activity of the abnormal β-chain was nearly three times that of ßA. The rate of synthesis of the β-chain of Hb Abraham Lincoln appeared to be approximately half that of the β-chain of Hb A.