Anderson P M, Desnick R J
Enzyme. 1982;28(2-3):146-57. doi: 10.1159/000459098.
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.
胆色素原（PBG）脱氨酶催化由四个胆色素原分子形成线性四吡咯——羟甲基胆素。该四吡咯要么由尿卟啉原合酶转化为尿卟啉原III，要么非酶促环化为尿卟啉原I。已开发出用于生物合成克级量胆色素原以及测定各种组织中PBG脱氨酶比活性的方法。使用该检测方法，测定了人红细胞、脑、肝脏、培养的成纤维细胞和淋巴细胞以及各种大鼠组织中的PBG脱氨酶活性作为参考值。此外，还确定了该检测方法用于急性间歇性卟啉症杂合子酶诊断的条件。
