Protein quality control during erythropoiesis and hemoglobin synthesis

E Khandros, MJ Weiss - Hematology/Oncology Clinics, 2010 - hemonc.theclinics.com
Hemoglobin synthesis during erythroid maturation is tightly coordinated to maximize the
production of functional hemoglobin A (a2b2) and to balance the levels of individual globin
subunits, which are unstable and cytotoxic in the absence of their partners. The importance
of this homeostasis is illustrated in b-thalassemia, a common inherited anemia in which b-
globin gene mutations cause a relative excess of free a-globin, which forms intracellular
precipitates and reactive oxygen species. 1 Accumulation of free a chains destroys erythroid …