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CHAPTER OUTLINE Synthesis of Porphyrins and Heme Regulation of Heme Biosynthesis High-Yield Terms Porphyrin: a group of aromatic organic compounds composed of 4 modified pyrrole subunits interconnected at their -carbon atoms via methine bridges Porphyria: any of a group of rare inherited or acquired disorders of enzymes involved in the production of the porphyrins and heme Hemin: protoporphyrin IX containing a ferric (Fe 3 + ) iron, as opposed to heme which contains ferrous (Fe 2 + ) iron Reticuloendothelial cells: a collective term for cells of the immune system that primarily comprises macrophages and monocytes but may include all phagocytic cells Bilirubin: open chain yellowish breakdown product of heme catabolism Kernicterus: bilirubin-induced brain dysfunction, also called bilirubin encephalopathy Sideroblastic anemia: a disease in which the bone marrow produces ringed sideroblasts rather than healthy erythrocytes, sideroblasts are abnormal nucleated erythroblasts with granules of iron in perinuclear mitochondria Porphyrins and Heme The porphyrins (Figure 33-1) are a group of aromatic organic compounds composed of 4 modified pyrrole subunits interconnected at their -carbon atoms via methine bridges (=CH)

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[4] Brown, R

R., Hunt, P