Aminolevulinic acid synthase

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{{Protbox start

  |Name=Aminolevulinic acid synthase
  |Photo=Heme synth step1.png
  |Caption=Heme synthesis
  |Gene=HUGO code: ALAS1, ALAS2
  |Structure= 
  |Review= stabilization

}} Template:Protbox finish The rate-limiting enzyme in porphyrin and heme biosynthesis is ALA synthase, the enzyme (Template:EC number) 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.

External links

• NIH