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