Protein synthesis in a cell free human reticulocyte system: ribosome function in thalassemia.

Abstract

A cell free system for protein synthesis was developed from peripheral erythroid cells of human subjects with thalassemia-major and with other chronic hemolytic anemias. The endogenous capacity to incorporate amino acids of ribosomes prepared from cells of thalassemic subjects is markedly diminished compared to that of non-thalassemic subjects. The ability of the ribosomes to respond to polyuridylic acid-directed phenylalanine incorporation is comparable for both groups. The defect in endogenous activity of ribosomes from cells of thalassemic subjects is not corrected in the cell free system by the addition of supernatant fractions prepared from cells of non-thalassemic subjects or from rabbit reticulocytes. The primary defect in thalassemia-major may involve a decrease or defect in messenger ribo-nucleic-acid (RNA) for hemoblogin A.