Porphobilinogen deaminase: methods and principles of the enzymatic assay.

  title={Porphobilinogen deaminase: methods and principles of the enzymatic assay.},
  author={Paul M. Anderson and Robert J Desnick},
  volume={28 2-3},
Porphobilinogen (PBG) deaminase catalyzes the formation of the linear tetrapyrrole, hydroxymethylbilane, from four molecules of PBG. The tetrapyrrole is either converted to uroporphyrinogen III by uroporphyrinogen cosynthetase or nonenzymatically cyclized to uroporphyrinogen I. Methods for the biosynthesis of gram quantities of PBG and for the determination of PBG-deaminase specific activity in various tissues were developed. Using this assay, the PBG-deaminase activities in human erythrocytes… CONTINUE READING