The Enzyme Database

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Accepted name: uroporphyrinogen-III synthase
Reaction: hydroxymethylbilane = uroporphyrinogen III + H2O
For diagram of the early stages of porphyrin biosynthesis, click here and for mechanism of reaction, click here
Other name(s): porphobilinogenase; uroporphyrinogen isomerase; uroporphyrinogen III cosynthase; URO-synthase; hydroxymethylbilane hydro-lyase (cyclizing)
Systematic name: hydroxymethylbilane hydro-lyase (cyclizing; uroporphyrinogen-III-forming)
Comments: In the presence of EC, hydroxymethylbilane synthase, the enzyme forms uroporphyrinogen III from porphobilinogen.
Links to other databases: BRENDA, EXPASY, KEGG, MetaCyc, PDB, CAS registry number: 37340-55-9
1.  Battersby, A.R., Fookes, C.J.R., Matcham, G.W.J. and McDonald, E. Biosynthesis of the pigments of life: formation of the macrocycle. Nature 285 (1980) 17–21. [PMID: 6769048]
2.  Tsai, S.-F., Bishop, D.F. and Desnick, R.J. Purification and properties of uroporphyrinogen III synthase from human erythrocytes. J. Biol. Chem. 262 (1987) 1268–1273. [PMID: 3805019]
[EC created 1982]

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