Porphyrin Biosynthesis (early stages)

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© IUBMB 2003


For nomenclature of tetrapyrroles, click here.

In the reaction of EC 4.2.1.24, the substrate forms an imine with the amino group of a lysine residue of the enzyme.

In the absence of EC 4.2.1.75, which cyclizes hydroxymethylbilane with reversal of ring D, cyclization occurs spontaneously, without such reversal, forming uroporphyrinogen I.


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porphyrin biosynthesis (later stages)
corrin biosynthesis
EC 1.2.1.70 glutamate-tRNA reductase
EC 2.3.1.37 5-aminolevulinate synthase
EC 2.5.1.61 hydroxymethylbilane synthase
EC 4.2.1.24 porphobilinogen synthase
EC 4.2.1.75 uroporphyrinogen-III synthase (mechanism)
EC 5.4.3.8 glutamate-1-semialdehyde 2,1-aminomutase(mechanism)
EC 6.1.1.17 glutamate—tRNA ligase