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...

Descripción completa

Guardado en:
Detalles Bibliográficos
Autores principales: Del C. Ríos de Molina, M., de Calmanovici, R.W., Grinstein, M., de Viale, L.C.S.M.
Formato: JOUR
Materias:
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
Acceso en línea:http://hdl.handle.net/20.500.12110/paper_00098981_v108_n3_p447_DelCRiosdeMolina
Aporte de:
Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA) de Universidad de Buenos Aires
id todo:paper_00098981_v108_n3_p447_DelCRiosdeMolina
record_format dspace
spelling todo:paper_00098981_v108_n3_p447_DelCRiosdeMolina2023-10-03T14:08:43Z Erythrocyte porphyrinogen carboxy-lyase activity in porphyria cutanea tarda and certain other human porphyrias Del C. Ríos de Molina, M. de Calmanovici, R.W. Grinstein, M. de Viale, L.C.S.M. 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. JOUR info:eu-repo/semantics/openAccess http://creativecommons.org/licenses/by/2.5/ar 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
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
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.
format JOUR
author Del C. Ríos de Molina, M.
de Calmanovici, R.W.
Grinstein, M.
de Viale, L.C.S.M.
author_facet Del C. Ríos de Molina, M.
de Calmanovici, R.W.
Grinstein, M.
de Viale, L.C.S.M.
author_sort Del C. Ríos de Molina, M.
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
url http://hdl.handle.net/20.500.12110/paper_00098981_v108_n3_p447_DelCRiosdeMolina
work_keys_str_mv AT delcriosdemolinam erythrocyteporphyrinogencarboxylyaseactivityinporphyriacutaneatardaandcertainotherhumanporphyrias
AT decalmanovicirw erythrocyteporphyrinogencarboxylyaseactivityinporphyriacutaneatardaandcertainotherhumanporphyrias
AT grinsteinm erythrocyteporphyrinogencarboxylyaseactivityinporphyriacutaneatardaandcertainotherhumanporphyrias
AT devialelcsm erythrocyteporphyrinogencarboxylyaseactivityinporphyriacutaneatardaandcertainotherhumanporphyrias
_version_ 1782027847953547264

Ejemplares similares