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Description

ALA exits the mitochondria, and the next cytoplasmic steps involve pairwise condensation of two ALA molecules by ALA dehydratase (PBGS) to form porphobilinogen (PBG), followed by tetrapyrrole assembly: PBG deaminase (HMBS) polymerizes four PBG units to hydroxymethylbilane (HMB), and uroporphyrinogen III synthase (UROS) cyclizes HMB to uroporphyrinogen III (with asymmetric orientation of the D ring)