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© IUBMB 2003
In the reaction of EC 126.96.36.199, the substrate forms an imine with the amino group of a lysine residue of the enzyme.
In the absence of EC 188.8.131.52, which cyclizes hydroxymethylbilane with reversal of ring D, cyclization occurs spontaneously, without such reversal, forming uroporphyrinogen I.
porphyrin biosynthesis (later stages)
EC 184.108.40.206 glutamate-tRNA reductase
EC 220.127.116.11 5-aminolevulinate synthase
EC 18.104.22.168 hydroxymethylbilane synthase
EC 22.214.171.124 porphobilinogen synthase
EC 126.96.36.199 uroporphyrinogen-III synthase (mechanism)
EC 188.8.131.52 glutamate-1-semialdehyde 2,1-aminomutase(mechanism)
EC 184.108.40.206 glutamatetRNA ligase