Luo J, Lim C K
Division of Clinical Cell Biology, MRC Clinical Research Centre, Harrow, Middlesex, U.K.
Biochem J. 1990 Jun 1;268(2):513-5. doi: 10.1042/bj2680513.
The isomeric composition of type-III heptacarboxylic porphyrinogens derived from decarbosylation of uroporphyrinogen III by erythrocyte uroporphyringogen decarboxylase was analysed by h.p.l.c. with electrochemical detection. All four possible isomers were identified, and there were little differences in the proportion of isomers formed by erythrocytes from normal subjects and from patients with sporadic porphyria cutanea tarda. The results provide conclusive evidence that the normal decarboxylation pathway is random in nature, and the fourth isomer only increases when enzyme abnormality is found.
采用高效液相色谱-电化学检测法分析了由红细胞尿卟啉原脱羧酶使尿卟啉原III脱羧生成的III型七羧基卟啉原的异构体组成。鉴定出了所有四种可能的异构体,正常受试者和散发性迟发性皮肤卟啉症患者红细胞形成的异构体比例几乎没有差异。结果提供了确凿证据,表明正常脱羧途径本质上是随机的,只有在发现酶异常时第四种异构体才会增加。