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