Heme and erythropoieis: more than a structural role.

摘要: Erythropoiesis is the biological process that consumes highest amount of body iron for heme synthesis. Heme synthesis in erythroid cells finely coordinated with alpha (α) and beta (β)-globin, resulting production hemoglobin, a tetramer 2α- 2β-globin chains, as prosthetic group. not only structural component but it plays multiple regulatory roles during differentiation precursors since controls its own regulates expression several erythroid-specific genes. synthesized developing progenitors by stage proerythroblast, through series eight enzymatic reactions divided between mitochondria cytosol. Defects lineage result sideroblastic anemias, characterized microcytic anemia associated to mitochondrial overload, or erythropoietic porphyrias, porphyrin deposition cells. Here, we focus on biosynthetic pathway human disorders due defective The role discussed well heme-mediated mechanisms allow orchestration adaptive cell response deficiency.