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Abstract:

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.

Registro:

Documento: Artículo
Título:Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias
Autor:Del C. Ríos de Molina, M.; de Calmanovici, R.W.; Grinstein, M.; de Viale, L.C.S.M.
Filiación:Laboratorio de Porfirinas, Departamento de Química Biológica, Facultad de Ciencias Exactas y Naturales, Ciudad Universitaria Nuñez, 1428 Buenos Aires, Argentina
Palabras clave:enzyme; blood and hemopoietic system; congenital erythropoietic porphyria; erythrocyte; human cell; major clinical study; normal human; porphyria cutanea tarda; porphyria variegata; porphyrinogen carboxylase; Carboxy-Lyases; Erythrocytes; Female; Human; Male; Porphyria; Porphyrinogens; Support, Non-U.S. Gov't; Uroporphyrinogen Decarboxylase
Año:1980
Volumen:108
Número:3
Página de inicio:447
Página de fin:456
DOI: http://dx.doi.org/10.1016/0009-8981(80)90352-6
Título revista:Clinica Chimica Acta
Título revista abreviado:Clin. Chim. Acta
ISSN:00098981
CODEN:CCATA
CAS:Carboxy-Lyases, EC 4.1.1.; Porphyrinogens; Uroporphyrinogen Decarboxylase, EC 4.1.1.37
Registro:https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_00098981_v108_n3_p447_DelCRiosdeMolina

Referencias:

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Citas:

---------- APA ----------
Del C. Ríos de Molina, M., de Calmanovici, R.W., Grinstein, M. & de Viale, L.C.S.M. (1980) . Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias. Clinica Chimica Acta, 108(3), 447-456.
http://dx.doi.org/10.1016/0009-8981(80)90352-6
---------- CHICAGO ----------
Del C. Ríos de Molina, M., de Calmanovici, R.W., Grinstein, M., de Viale, L.C.S.M. "Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias" . Clinica Chimica Acta 108, no. 3 (1980) : 447-456.
http://dx.doi.org/10.1016/0009-8981(80)90352-6
---------- MLA ----------
Del C. Ríos de Molina, M., de Calmanovici, R.W., Grinstein, M., de Viale, L.C.S.M. "Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias" . Clinica Chimica Acta, vol. 108, no. 3, 1980, pp. 447-456.
http://dx.doi.org/10.1016/0009-8981(80)90352-6
---------- VANCOUVER ----------
Del C. Ríos de Molina, M., de Calmanovici, R.W., Grinstein, M., de Viale, L.C.S.M. Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias. Clin. Chim. Acta. 1980;108(3):447-456.
http://dx.doi.org/10.1016/0009-8981(80)90352-6