Abstract
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.
Full text
PDFSelected References
These references are in PubMed. This may not be the complete list of references from this article.
- Jackson A. H., Sancovich H. A., Ferramola A. M., Evans N., Games D. E., Matlin S. A., Elder G. H., Smith S. G. Macrocyclic intermediates in the biosynthesis of porphyrins. Philos Trans R Soc Lond B Biol Sci. 1976 Feb 5;273(924):191–206. doi: 10.1098/rstb.1976.0009. [DOI] [PubMed] [Google Scholar]
- Kawanishi S., Seki Y., Sano S. Uroporphyrinogen decarboxylase. Purification, properties, and inhibition by polychlorinated biphenyl isomers. J Biol Chem. 1983 Apr 10;258(7):4285–4292. [PubMed] [Google Scholar]
- Kushner J. P., Barbuto A. J., Lee G. R. An inherited enzymatic defect in porphyria cutanea tarda: decreased uroporphyrinogen decarboxylase activity. J Clin Invest. 1976 Nov;58(5):1089–1097. doi: 10.1172/JCI108560. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Lim C. K., Li F., Peters T. J. High-performance liquid chromatography of type-III heptocarboxylic porphyrinogen isomers. Biochem J. 1987 Oct 1;247(1):229–232. doi: 10.1042/bj2470229. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Lim C. K., Rideout J. M., Wright D. J. High-performance liquid chromatography of naturally occurring 8-, 7-, 6-, 5- and 4-carboxylic porphyrin isomers. J Chromatogr. 1983 Dec 30;282:629–641. doi: 10.1016/s0021-9673(00)91640-6. [DOI] [PubMed] [Google Scholar]
- Lim C. K., Rideout J. M., Wright D. J. Separation of porphyrin isomers by high-performance liquid chromatography. Biochem J. 1983 May 1;211(2):435–438. doi: 10.1042/bj2110435. [DOI] [PMC free article] [PubMed] [Google Scholar]
- MAUZERALL D., GRANICK S. Porphyrin biosynthesis in erythrocytes. III. Uroporphyrinogen and its decarboxylase. J Biol Chem. 1958 Jun;232(2):1141–1162. [PubMed] [Google Scholar]
- Straka J. G., Kushner J. P. Purification and characterization of bovine hepatic uroporphyrinogen decarboxylase. Biochemistry. 1983 Sep 27;22(20):4664–4672. doi: 10.1021/bi00289a009. [DOI] [PubMed] [Google Scholar]
- Tomio J. M., García R. C., San Martín de Viale L. C., Grinstein M. Porphyrin biosynthesis. VII. Porphyrinogen carboxy-lyase from avian erythrocytes. Purification and properties. Biochim Biophys Acta. 1970 Feb 11;198(2):353–363. doi: 10.1016/0005-2744(70)90068-9. [DOI] [PubMed] [Google Scholar]