Unbalanced globin chain synthesis by Hb Lincoln Park (anti-Lepore) reticulocytes.
Hemoglobin synthesis was studied in vitro in reticulocytes from a patient with the anti-Lepore variant Hb Lincoln Park. Incorporation of L-leucine-3H into the alpha and beta delta chains of Hb Lincoln Park was substantially less than the incorporation into the corresponding globin chains of Hb A, with the rate of synthesis of the beta delta chain being similar to that of the delta chain of Hb A2. Synthesis of the alpha and total non-alpha globin components was unbalanced, with a substantial excess of alpha chain synthesis. These findings help to explain the mild hemolytic disease present in individuals with this hemoglobin variant.
D005914:Globins / Q000096:biosynthesis* / Q000378:metabolism; D006441:Hemoglobin A / Q000096:biosynthesis; D006455:Hemoglobins, Abnormal; D006801:Humans; D007930:Leucine / Q000378:metabolism; D012156:Reticulocytes / Q000378:metabolism*
D006455:Hemoglobins, Abnormal; D005914:Globins; D006441:Hemoglobin A; D007930:Leucine