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Updated: Jun 25, 2026

Measurement of Heme Synthesis Levels in Mammalian Cells
Published on: July 9, 2015
5-aminolevulinate synthase: catalysis of the first step of heme biosynthesis
1Department of Molecular Medicine, College of Medicine, University of South Florida, Tampa, Florida 33612, USA.
Abstract:
5-Aminolevulinate synthase is a homodimeric pyridoxal 5'-phosphate-dependent enzyme that catalyzes the first step of the heme biosynthetic pathway in animals, fungi, and the alpha-subclass of the photosynthetic purple bacteria. The reaction cycle involves condensation of glycine with succinyl-coenzyme A to yield 5-aminolevulinate, carbon dioxide, and CoA. Mutations in the human erythroid-specific aminolevulinate synthase gene are associated with the erythropoietic disorder X-linked sideroblastic anemia. Recent kinetic and crystallographic data have facilitated an unprecedented understanding of how this important enzyme produces 5-aminolevulinate, and suggest possible directions for future research that may lead to treatments not only for X-linked sideroblastic anemia, but also other diseases.
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