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δ-Aminolevulinic acid synthetase (ALAS)

Target: ALAS
Molecular classification: Enzyme, Transferase, Acyltransferase

Overview

δ-Aminolevulinic acid synthetase (ALAS) is a mitochondrial enzyme that catalyzes the first and rate-limiting step in the heme biosynthetic pathway. It condenses glycine and succinyl-CoA to form δ-aminolevulinic acid (ALA). There are two main isoforms: ALAS1 (housekeeping) and ALAS2 (erythroid-specific). Mutations or dysregulation can lead to disorders such as sideroblastic anemia or porphyrias due to impaired heme synthesis pathways.

Mechanism of action

Inhibition of δ-aminolevulinic acid synthesis

Biological functions

Heme synthesisPorphyrin biosynthesis

Disease associations

Sideroblastic anemiaPorphyria

Safety considerations

Accumulation of porphyrin precursorsDrug-induced porphyria

Biomarkers

ALA levelsPorphyrin levels

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