Monteiro H P, Bechara E J, Abdalla D S
Fundaçao Hemocentro de Sao Paulo-F/HSP, Brazil.
Mol Cell Biochem. 1991 Apr 24;103(1):73-83. doi: 10.1007/BF00229595.
Porphyrias are inherited and acquired diseases of erythroid or hepatic origin, in which there are defects in specific enzymes of the heme biosynthetic pathway. In patients with intermittent acute porphyria and lead poisoning the erythrocytic activities of superoxide dismutase and glutathione peroxidase are reported to be increased. Our studies demonstrated that d-aminolevulinic acid, a heme precursor accumulated in both diseases, undergoes enolization at pH less than 7.0 before it autoxidizes. The autoxidation of d-aminolevulinic acid, in the presence or absence of oxyhemoglobin has been proposed as a source of oxy and carbon-centred radicals in the cells of intermittent acute porphyria and saturnism carriers. Thus, the increased levels of antioxidant enzymes can be viewed as an intracellular response against the deleterious effects of these extremely reactive species.
卟啉病是起源于红细胞或肝脏的遗传性和后天性疾病，其中血红素生物合成途径的特定酶存在缺陷。据报道，在间歇性急性卟啉病和铅中毒患者中，超氧化物歧化酶和谷胱甘肽过氧化物酶的红细胞活性会增加。我们的研究表明，δ-氨基乙酰丙酸是这两种疾病中积累的血红素前体，在自氧化之前，在pH小于7.0时会发生烯醇化。在有或没有氧合血红蛋白存在的情况下，δ-氨基乙酰丙酸的自氧化被认为是间歇性急性卟啉病和铅中毒携带者细胞中氧自由基和碳中心自由基的来源。因此，抗氧化酶水平的升高可被视为细胞对这些极具反应性的物质的有害影响的一种细胞内反应。
