5 aminolevulinic acid
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5-Aminolevulinic acid
It is formed by the condensation of glycine and succinite (succinyl-CoA) via the intermediate adipate (2-amino-3-oxo-1,6-hexanedioic acid), which is subsequently decarboxylated (removes CO2 ) to form 5-aminolevulinic acid.
The reaction is catalyzed by aminolevulate synthase (ALA-synthase, ALAS), located in the mitochondrial matrix. Pyridoxal-5-phosphate acts as a cofactor, forming a temporary Schiff base with glycine. Enzyme activity is sensitive to vitamine B6 deficiency. 5-aminolevulinic acid is the starting precoursor in the metabolism of heme.
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References
- MATOUŠ, Bohuslav, et al. Základy lekárskej chemie a biochemie. 2010. edition. Praha : Galen, 2010. 0 pp. ISBN 978-80-7262-702-8.
