Porphobilinogen deaminase

Definition:

As part of the heme biosynthetic pathway, catalyzes the sequential polymerization of four molecules of porphobilinogen to form hydroxymethylbilane, also known as preuroporphyrinogen. Catalysis begins with the assembly of the dipyrromethane cofactor by the apoenzyme from two molecules of porphobilinogen or from preuroporphyrinogen. The covalently linked cofactor acts as a primer, around which the tetrapyrrole product is assembled. In the last step of catalysis, the product, preuroporphyrinogen, is released, leaving the cofactor bound to the holodeaminase intact.

References:

[1]. Gaojie Song, et al. Structural insight into acute intermittent porphyria. FASEB J. 2009 Feb;23(2):396-404. [Content Brief]

[2]. Dana Ulbrichova, et al. Correlation between biochemical findings, structural and enzymatic abnormalities in mutated HMBS identified in six Israeli families with acute intermittent porphyria. Blood Cells Mol Dis. 2009 Mar-Apr;42(2):167-73. [Content Brief]

[3]. Helene J Bustad, et al. Conformational stability and activity analysis of two hydroxymethylbilane synthase mutants, K132N and V215E, with different phenotypic association with acute intermittent porphyria. Biosci Rep. 2013 Aug 8;33(4):e00056. [Content Brief]

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