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Delta-aminolevulinic Acid Synthase (ALAS)

Target: ALAS
Molecular classification: Enzyme

Overview

Delta-aminolevulinic acid synthase (ALAS) is a mitochondrial enzyme that catalyzes the first and rate-limiting step in the heme biosynthesis pathway. It condenses glycine and succinyl-CoA to form delta-aminolevulinic acid (ALA). There are two main isoforms: ALAS1 (ubiquitous) and ALAS2 (erythroid-specific). ALAS requires pyridoxal 5'-phosphate (PLP) as a cofactor and is regulated by feedback inhibition by heme and other mechanisms. Mutations in ALAS2 can lead to sideroblastic anemia.

Mechanism of action

Inhibition of enzymatic activity (potential)

Biological functions

Heme biosynthesisCatalysis

Disease associations

Sideroblastic anemia

Safety considerations

Potential for porphyrin accumulationPotential for heme deficiency

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