Porphobilinogen deaminase: Methods and principles of the enzymatic assay

P. M. Anderson; R. J. Desnick

doi:10.1159/000459098

Porphobilinogen deaminase: Methods and principles of the enzymatic assay

P. M. Anderson, R. J. Desnick

Pediatrics

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39 Scopus citations

Abstract

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, brain, liver and cultured fibroblasts and lymphoblasts as well as various rat tissues were determined as reference values. In addition, the conditions for the use of this assay in the enzymatic diagnosis of heterozygotes for acute intermittent porphyria were established.

Original language	English (US)
Pages (from-to)	146-156
Number of pages	11
Journal	Enzyme
Volume	28
Issue number	2-3
DOIs	https://doi.org/10.1159/000459098
State	Published - 1982

ASJC Scopus subject areas

Biochemistry

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abstract = "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, brain, liver and cultured fibroblasts and lymphoblasts as well as various rat tissues were determined as reference values. In addition, the conditions for the use of this assay in the enzymatic diagnosis of heterozygotes for acute intermittent porphyria were established.",

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AB - 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, brain, liver and cultured fibroblasts and lymphoblasts as well as various rat tissues were determined as reference values. In addition, the conditions for the use of this assay in the enzymatic diagnosis of heterozygotes for acute intermittent porphyria were established.

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Porphobilinogen deaminase: Methods and principles of the enzymatic assay

Abstract

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