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