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Porphobilinogen Deaminase

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3eq1, resolution 2.80Å (default scene)
Ligands: DPM, SO4
Activity: Hydroxymethylbilane synthase, with EC number 2.5.1.61
Resources: FirstGlance, OCA, RCSB, PDBsum
Coordinates: save as pdb, mmCIF, xml


Porphobilinogen deaminase (PBGD) also known as Hydroxymethylbilane synthase, is the third enzyme in the heme biosynthesis pathways in mammals[1]. It catalyses the polymerization of four porphobilinogen molecules to yield hydroxymethylbilane[2]

Function

Structure

Dipyrromethane

References

  1. ↑ Gill R, Kolstoe SE, Mohammed F, Al D-Bass A, Mosely JE, Sarwar M, Cooper JB, Wood SP, Shoolingin-Jordan PM. Structure of human porphobilinogen deaminase at 2.8 A: the molecular basis of acute intermittent porphyria. Biochem J. 2009 Apr 28;420(1):17-25. PMID:19207107 doi:10.1042/BJ20082077
  2. ↑ Lopes MG, Pereirinha A, De Padua F. Intraventricular conduction defects associated with hypertrophic myocardiopathy-an echocardiographic and vectorcardiographic study. Adv Cardiol. 1978;21:223-7. doi: 10.1159/000400454. PMID:145793 doi:https://dx.doi.org/10.1159/000400454