Aminolevulinic acid synthase

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Heme_synth_step1.png
ALA synthase
The rate-limiting enzyme in porphyrin and heme biosynthesis is ALA synthase, the enzyme that catalyses glycine and succinyl-CoA into D-Aminolevulinic acid. In humans, transcription of ALA synthase is tightly controlled by the presence of Fe2+-binding elements, to prevent accumulation of porphyrin intermediates in the absence of iron.

ALA synthase removes the carboxyl group from glycine and the CoA from the succinate, forming δ-amino levulinic acid (dALA), so called because the amino group is on the fourth carbon atom in the molecule.

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