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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1980
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LEADER | 10204caa a22009377a 4500 | ||
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001 | PAPER-1306 | ||
003 | AR-BaUEN | ||
005 | 20240904173203.0 | ||
008 | 190411s1980 xx ||||fo|||| 00| 0 eng|d | ||
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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