6 Heme SynthesisHeme is synthesized in a complex series of steps involving enzymes in the mitochondrion and in the cytosol of the cellThe first step in heme synthesis takes place in the mitochondrion, with the condensation of succinyl CoA and glycine by ALA synthase to form 5-aminolevulic acid (ALA)
7 This molecule is transported to the cytosol where a series of reactions produce a ring structure called coproporphyrinogen IIIThis molecule returns to the mitochondrion where an addition reaction produces protoporhyrin IXThe enzyme ferrochelatase inserts iron into the ring structure of protoporphyrin IX to produce heme
8 Heme Biosynthesis. The sythesis of heme is a complex process that involves multiple enzymatic steps. The process begins in the mitochondrion with the condensation of succinyl-CoA and glycine to form 5-aminolevulinic acid. A series of steps in the cytoplasm produce coproporphrynogen III, which re-enters the mitochondrion. The final enzymatic steps produce heme.
12 2 Suksinil-KoA + 2 Glisin membentuk senyawa Pirol 4 Pirol akan membentuk senyawa Protoporfirin IX Protoporfirin IX + Fe2+ membentuk senyawa Heme 4 Hem + Polipeptida membentuk Rantai Hemoglobin (α atau β) Rantai 2α + Rantai 2β membentuk hemoglobin A