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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| Acceso en línea: | https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_00098981_v108_n3_p447_DelCRiosdeMolina http://hdl.handle.net/20.500.12110/paper_00098981_v108_n3_p447_DelCRiosdeMolina |
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paper:paper_00098981_v108_n3_p447_DelCRiosdeMolina2023-06-08T14:34:04Z Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias Ríos de Molina, María del Carmen Grinstein, Moisés 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 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. Fil:Del C. Ríos de Molina, M. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Grinstein, M. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. 1980 https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_00098981_v108_n3_p447_DelCRiosdeMolina http://hdl.handle.net/20.500.12110/paper_00098981_v108_n3_p447_DelCRiosdeMolina |
| institution |
Universidad de Buenos Aires |
| institution_str |
I-28 |
| repository_str |
R-134 |
| collection |
Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA) |
| topic |
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 |
| spellingShingle |
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 Ríos de Molina, María del Carmen Grinstein, Moisés Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias |
| topic_facet |
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 |
| description |
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. |
| author |
Ríos de Molina, María del Carmen Grinstein, Moisés |
| author_facet |
Ríos de Molina, María del Carmen Grinstein, Moisés |
| author_sort |
Ríos de Molina, María del Carmen |
| title |
Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias |
| title_short |
Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias |
| title_full |
Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias |
| title_fullStr |
Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias |
| title_full_unstemmed |
Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias |
| title_sort |
erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias |
| publishDate |
1980 |
| url |
https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_00098981_v108_n3_p447_DelCRiosdeMolina http://hdl.handle.net/20.500.12110/paper_00098981_v108_n3_p447_DelCRiosdeMolina |
| work_keys_str_mv |
AT riosdemolinamariadelcarmen erythrocyteporphyrinogencarboxylyaseactivityinporphyriacutaneatardaandcertainotherhumanporphyrias AT grinsteinmoises erythrocyteporphyrinogencarboxylyaseactivityinporphyriacutaneatardaandcertainotherhumanporphyrias |
| _version_ |
1768542968752373760 |