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