Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias

Red cell porphyrinogen carboxy-lyase activity was measured using uroporphyrinogen III as substrate in 18 normal persons, 7 male patients with porphyria cutanea tarda, 3 female patients with erythropoietic protoporphyria and 2 female patients with variegate porphyria. The mean values obtained in norm...

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Detalles Bibliográficos
Autor principal: Ríos de Molina, María del Carmen
Otros Autores: de Calmanovici, R.W, Grinstein, M., de Viale, L.C.S.M
Formato: Capítulo de libro
Lenguaje:Inglés
Publicado: 1980
Acceso en línea:Registro en Scopus
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Registro en la Biblioteca Digital
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024 7 |2 scopus  |a 2-s2.0-0019134395 
024 7 |2 cas  |a Carboxy-Lyases, EC 4.1.1.; Porphyrinogens; Uroporphyrinogen Decarboxylase, EC 4.1.1.37 
040 |a Scopus  |b spa  |c AR-BaUEN  |d AR-BaUEN 
030 |a CCATA 
100 1 |a Ríos de Molina, María del Carmen 
245 1 0 |a Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias 
260 |c 1980 
270 1 0 |m de Viale, L.C.S.M.; Laboratorio de Porfirinas, Departamento de Química Biológica, Facultad de Ciencias Exactas y Naturales, Ciudad Universitaria Nuñez, 1428 Buenos Aires, Argentina 
506 |2 openaire  |e Política editorial 
504 |a Batlle A.M. del, Grinstein, Porphyrin biosynthesis Phyriaporphyrinogen III a normal intermediate in the biosynthesis of protoporphyrin 9 (1962) Biochimica et Biophysica Acta, 62, pp. 197-200 
504 |a San Martin de Viale, Grinstein, Porphyrin biosynthesis IV 5- and 6-COOH porphyrinogens (Type III) as normal intermediates in haem biosynthesis (1968) Biochimica et Biophysica Acta (BBA) - General Subjects, 158, pp. 79-91 
504 |a Tomio, García, San Martin de Viale, Grinstein, Porphyrin biosynthesis VII Porphyrinogen carboxy-lyase from avian erythrocytes Purification and properties (1970) Biochimica et Biophysica Acta (BBA) - Enzymology, 198, pp. 353-363 
504 |a Garcia, San Martin de Viale, Tomio, Grinstein, Porphyrin biosynthesis X Porphyrinogen carboxy-lyase from avian erythrocytes Further properties (1973) Biochimica et Biophysica Acta (BBA) - Enzymology, 309, pp. 203-210 
504 |a Jackson, Sancovich, Ferramola, Evans, Games, Matlin, Eider, Smith, Macrocyclic intermediates in the biosynthesis of porphyrins (1976) Philosophical Transactions of the Royal Society B: Biological Sciences, 273, pp. 191-206 
504 |a San Martín de Viale, Aragonés, Tomio, Tetrapyrroles as substrates and inhibitors of Porphyrinogen carboxy-lyase from rat liver (1976) Acta Physiol Lat Am, 26, pp. 403-414 
504 |a San Martin de Viale, Viale, Nacht, Grinstein, Experimental porphyria induced in rats by hexachlorobenzene Study of the porphyrins excreted by urine (1970) Clinica Chimica Acta, 28, pp. 13-23 
504 |a Grinstein, Schwartz, Watson, Studies of the uroporphyrins. The purification of uroporphyrin I and the nature of the Waldenström's uroporphyrin, as isolated from porphyria material (1945) J. Biol. Chem., 157, pp. 323-343 
504 |a Nacht, San Martín de Viale, Grinstein, Human porphyria cutanea tarda Isolation and properties of the urinary porphyrins (1970) Clinica Chimica Acta, 27, pp. 445-452 
504 |a Dowdle, Goldswain, Spong, Eales, The pattern of porphyrin isomer accumulation and excretion in symptomatic porphyria (1970) Clin Sci, 39, pp. 147-158 
504 |a Ríos de Molina, Wainstok de Calmanovici, Tomio, San Martin de Viale, Porfiria inducida en ratas por hexaclorobenceno. Accion de la droga sobre el contenido de porfirinas y la actividad de la uroporfirinógeno decarboxilasa en distintos órganos (1975) An. Asoc. Quim. Argent., 63, pp. 313-332 
504 |a San Martin de Viale, Rios de Molina M. del, Wainstok de Calmanovici, Tomio, Porphyrins and Porphyrinogen carboxy-lyase in hexachlorobenzene-induced porphyria (1977) Biochem J, 168, pp. 393-400 
504 |a Elder, Evans, Matlin, The effect of the porphyrogenic compound hexachlorobenzene on the activity of hepatic uroporphyrinogen decarboxylase in the rat (1976) Clin Sci Mol Med, 51, pp. 71-80 
504 |a Kushner, Barbuto, Lee, An inherited enzymatic defect in porphyria cutanea tarda Decreased uroporphyrinogen decarboxylase activity (1976) Journal of Clinical Investigation, 58, pp. 1089-1097 
504 |a Benedetto, Kushner, Taylor, Porphyria cutanea tarda in three generations of a single family (1978) New England Journal of Medicine, 298, pp. 358-362 
504 |a Blekkenhorst, Pimstone, Eales, Porphyria cutanea tarda in South Africa. Metabolic basis of disordered haem biosynthesis (1976) Porphyrins in Human Diseases, pp. 299-311. , M. Doss, Karger, Basel 
504 |a San Martín de Viale, Rios de Molina M. del, Wainstok de Calmanovici, Tomio, Experimental porphyria induced in rats by hexachlorobenzene. Studies on the stepwise decarboxylation of uroporphyrinogen and phyriaporphyrinogen in vivo and in vitro in several tissues (1976) Porphyrins in Human Diseases, pp. 445-452. , M. Doss, Karger, Basel 
504 |a Grinstein, Wintrobe, Spectrophotometric micromethod for the quantitative determination of the free erythrocyte protoporphyrin (1948) J Biol Chem, 172, pp. 459-467 
504 |a San Martin de Viale, Wainstok de Calmanovici, Ríos de Molina M. del, Grinstein, Studies on porphyrin biosynthesis in lead-intoxicated rabbits (1976) Clinica Chimica Acta, 69, pp. 375-382 
504 |a Mauzerall, Granick, The occurrence and determination of δ-aminolevulinic acid and porphobilinogen in urine (1956) J Biol Chem, 219, pp. 435-446 
504 |a Rimington, Sveinsson, The Spectrophotometric determination of uroporphyrin (1950) Scandinavian Journal of Clinical & Laboratory Investigation, 2, pp. 209-216 
504 |a Grinstein, Simplified method for the determination of porphyrins in body fluids (1977) Analytical Biochemistry, 77, pp. 577-580 
504 |a Schwartz, Berg, Bossenmaier, Dinsmore, (1960) Methods of Biochemical Analysis, 8, p. 257. , 3rd ed., D. Glick, Intersciences Publishers, New York 
504 |a Mauzerall, Granick, Porphyrin biosynthesis in erythrocytes III Uroporphyrinogen and its decarboxylase (1958) J Biol Chem, 232, pp. 1141-1162 
504 |a Aragonés, García, San Martin de Viale, Tomio, Grinstein, Estudios de la transformación de uroporfirinógenos en coproporfirinógenos por decarboxilasas de eritrocitos de polio e hígado de rata (1972) An. Asoc. Quim. Argent., 60, pp. 239-248 
504 |a Elder, Path, Lee, Tovey, Decreased activity of hepatic uroporphyrinogen decarboxylase in sporadic porphyria cutanea tarda (1978) New England Journal of Medicine, 299, pp. 274-278 
504 |a de Vemeuil, Aitken, Nordmann, Familial and sporadic porphyria cutanea (1978) Human Genetics, 44, pp. 145-151 
504 |a Cornford, Transformation of porphobilinogen into porphyrins by preparations from human erythrocytes (1964) Biochem J, 91, pp. 64-73 
504 |a Andersen, Sassa, Peterson, Kappas, Increased uroporphyrinogen I synthetase 6-aininolevulinic acid dehydratase and protoporphyrin in hemolytic anemias (1977) The American Journal of Medicine, 63, pp. 359-364 
520 3 |a Red cell porphyrinogen carboxy-lyase activity was measured using uroporphyrinogen III as substrate in 18 normal persons, 7 male patients with porphyria cutanea tarda, 3 female patients with erythropoietic protoporphyria and 2 female patients with variegate porphyria. The mean values obtained in normal subjects were 0.151 nmol of uroporphyrinogen disappeared in 30 min per mg of protein, and 0.038 nmol of coproporphyrinogen formed in 30 min per mg of protein. We have not been able to detect significant differences between males and females. In porphyria cutanea tarda the enzyme activity was the same as in normal subjects considering either substrate disappearance or end product formation. The differences were not significant at the p < 0.05 level. Patients with variegate porphyria also exhibited normal erythrocyte porphyrinogen carboxy-lyase activity. The enzyme activity of erythrocytes from patients with erythropoietic protoporphyria was higher than in normals; mean values for specific activities being 0.204 nmol of uroporphyrinogen disappeared, and 0.071 nmol of coproporphyrinogen formed. The significance of the results with respect to the chemical picture of different porphyrias is discussed. © 1980.  |l eng 
536 |a Detalles de la financiación: Consejo Nacional de Investigaciones Científicas y Técnicas 
536 |a Detalles de la financiación: Secretaria de Ciencia y Tecnica, Universidad de Buenos Aires 
536 |a Detalles de la financiación: ments. Part of this work was supported by research grants from Consejo National de Investigaciones Cientificas y T&nicas and Secretaria de Ciencia y Tbcnica (Argentina). 
593 |a Laboratorio de Porfirinas, Departamento de Química Biológica, Facultad de Ciencias Exactas y Naturales, Ciudad Universitaria Nuñez, 1428 Buenos Aires, Argentina 
690 1 0 |a ENZYME 
690 1 0 |a BLOOD AND HEMOPOIETIC SYSTEM 
690 1 0 |a CONGENITAL ERYTHROPOIETIC PORPHYRIA 
690 1 0 |a ERYTHROCYTE 
690 1 0 |a HUMAN CELL 
690 1 0 |a MAJOR CLINICAL STUDY 
690 1 0 |a NORMAL HUMAN 
690 1 0 |a PORPHYRIA CUTANEA TARDA 
690 1 0 |a PORPHYRIA VARIEGATA 
690 1 0 |a PORPHYRINOGEN CARBOXYLASE 
690 1 0 |a CARBOXY-LYASES 
690 1 0 |a ERYTHROCYTES 
690 1 0 |a FEMALE 
690 1 0 |a HUMAN 
690 1 0 |a MALE 
690 1 0 |a PORPHYRIA 
690 1 0 |a PORPHYRINOGENS 
690 1 0 |a SUPPORT, NON-U.S. GOV'T 
690 1 0 |a UROPORPHYRINOGEN DECARBOXYLASE 
700 1 |a de Calmanovici, R.W. 
700 1 |a Grinstein, M. 
700 1 |a de Viale, L.C.S.M. 
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