Mitochondrial ALA synthase (ALAS)
Cofactors used
Clinical / pharmacological. ALAS2 defects: X-linked sideroblastic anaemia (B6-responsive)
What it does, reaction by reaction
Heme Biosynthesis (Porphyrin Pathway) Amino Acid & Nitrogen Metabolism · Mitochondrion + cytosol
Glycine + succinyl-CoA → δ-aminolevulinate (ALA) + CO₂ + CoA
Converts Glycine Succinyl-CoA into delta--aminolevulinate (ALA) CO2 CoA-SH
› Notes
Mitochondrial ALA synthase (ALAS) requires PLP (vitamin B6). ALAS1 is the rate-limiting and committed step in nonerythroid cells and is feedback-inhibited at transcriptional, translational, and mitochondrial-import levels by heme/hemin; erythroid ALAS2 is rate-limited by iron-dependent translation through an iron-responsive element.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
No regulation recorded for this enzyme in the source documents.
Recent literature
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