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