Porphobilinogen synthase

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Template:STRUCTURE 1e51

Contents 1 Function 2 Disease 2.1 3D structures of porphobilinogen synthase 3 References

Function

Porphobilinogen synthase (PBS) catalyzes the formation of porphobilinogen (PBG) from 2 molecules of aminolevulinic acid (ALA). PBS participates in porphyrin biosynthesis^[1]. Levulinic acid (LA) is an inhibitor of PBS. Porphyrin is the precursor of hemes, chlorophyll and vitamin B12.

Disease

PBS deficiency can be caused by lead and other heavy metal poisoning^[2].

3D structures of porphobilinogen synthase

Updated on 10-July-2016

References

1. ↑ Jaffe EK. The porphobilinogen synthase catalyzed reaction mechanism. Bioorg Chem. 2004 Oct;32(5):316-25. PMID:15381398 doi:http://dx.doi.org/10.1016/j.bioorg.2004.05.010
2. ↑ Doss M, Muller WA. Acute lead poisoning in inherited porphobilinogen synthase (delta-aminolevulinic acid dehydrase) deficiency. Blut. 1982 Aug;45(2):131-9. PMID:7104498