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Aminolevulinate Synthase 1 (None)
- Target
- None
- Molecular classification
- Enzyme, Mitochondrial enzyme, PLP-dependent homodimer
01
Overview
Aminolevulinate synthase 1 (ALAS1) is a mitochondrial enzyme that catalyzes the first and rate-limiting step in the biosynthesis of heme. This step involves the condensation of glycine with succinyl-CoA to form delta-aminolevulinic acid (ALA). ALAS1 is considered a "housekeeping" isozyme expressed in most tissues except erythroid cells. Its activity is subject to feedback inhibition by heme. Mutations in ALAS2, another isozyme, cause X-linked sideroblastic anemia, while disruption of ALAS1 expression or function may contribute to metabolic disorders.
- Other names
- ALASALAS3ALASHMIG4ALAS-Hdelta-aminolevulinate synthase 1
02
Mechanism of action
Inhibition of heme biosynthesis
03
Biological functions
Heme biosynthesisCatalysisPorphyrin biosynthesis
04
Disease associations
Sideroblastic anemia (indirectly, via ALAS2)Metabolic disorders involving impaired energy metabolismAcute hepatic porphyrias
05
Safety considerations
Accumulation of porphyrin intermediates
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