Delta-aminolevulinate synthase catalyzes the condensation ofglycine withsuccinyl-CoA to formdelta-aminolevulinic acid. This nuclear-encodedmitochondrial enzyme is the first and rate-limiting enzyme in the mammalian heme biosynthetic pathway. There are 2 tissue-specific isozymes: a housekeeping enzyme encoded by the ALAS1 gene and an erythroid tissue-specific enzyme encoded byALAS2.[6]
Mice lacking this gene exhibit embryonic lethality, indicating that ALAS is essential for early embryogenesis.[7]
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^"Mouse PubMed Reference:".National Center for Biotechnology Information, U.S. National Library of Medicine.
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