Reference values of 5-Aminolevulinate dehydrase and Porphobilinogen deaminase in the Spanish population from Madrid

5-Aminolevulinate dehydrase (ALA-D) and porphobilinogen deaminase (PBG- D) are cytosolic enzymes involved in heme biosynthesis. ALA-D activity is altered both genetically and by the action of various environmental factors, including exposure to lead. The activity of PBG-D is reduced in acute intermi...

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Autores principales:	Santos, J.L., Fontanellas, A., Batlle, A.M.C., De Salamanca, R.E.
Formato:	JOUR
Materias:	Aminolevulinate dehydrase Porphobilinogen deaminase Reference range porphobilinogen deaminase porphobilinogen synthase adolescent adult aged article child cytosol enzyme activity enzyme analysis female heme synthesis human lead poisoning male normal human porphyria reference value Spain
Acceso en línea:	http://hdl.handle.net/20.500.12110/paper_01476513_v39_n3_p168_Santos
Aporte de:	Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA) de Universidad de Buenos Aires

id	todo:paper_01476513_v39_n3_p168_Santos
record_format	dspace
spelling	todo:paper_01476513_v39_n3_p168_Santos2023-10-03T15:00:43Z Reference values of 5-Aminolevulinate dehydrase and Porphobilinogen deaminase in the Spanish population from Madrid Santos, J.L. Fontanellas, A. Batlle, A.M.C. De Salamanca, R.E. Aminolevulinate dehydrase Porphobilinogen deaminase Reference range porphobilinogen deaminase porphobilinogen synthase adolescent adult aged article child cytosol enzyme activity enzyme analysis female heme synthesis human lead poisoning male normal human porphyria reference value Spain 5-Aminolevulinate dehydrase (ALA-D) and porphobilinogen deaminase (PBG- D) are cytosolic enzymes involved in heme biosynthesis. ALA-D activity is altered both genetically and by the action of various environmental factors, including exposure to lead. The activity of PBG-D is reduced in acute intermittent porphyria. The aim of this work is to establish the 95% reference range of the erythrocytic activity of ALA-D and PBG-D in a control population. ALA-D activity limits were 15.8 and 50.2 nmol of PBG/ml of red blood cells (RBCS) per minute. For the activity of ALA-D restored by addition of zinc and dithiothreitol ('restored ALA-D'), these limits were 44.1 and 86.5 units. It has been found that the 'restored ALA-D'/ALA-D ratio is very useful for the evaluation of lead toxicity, and its 95% reference range was between 1.22 and 3.06. It has been demonstrated that the best method for measuring erythrocytic PBG-D is using PBG, but not ALA, as substrate; its 95% reference range was between 20.9 and 63.2 nmol of uroporphyrin/ml of RBCs per hour. Knowledge of these reference range values in a control population constitutes the basis for an accurate diagnosis of heavy metal intoxication and acute intermittent porphyria. JOUR info:eu-repo/semantics/openAccess http://creativecommons.org/licenses/by/2.5/ar http://hdl.handle.net/20.500.12110/paper_01476513_v39_n3_p168_Santos
institution	Universidad de Buenos Aires
institution_str	I-28
repository_str	R-134
collection	Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA)
topic	Aminolevulinate dehydrase Porphobilinogen deaminase Reference range porphobilinogen deaminase porphobilinogen synthase adolescent adult aged article child cytosol enzyme activity enzyme analysis female heme synthesis human lead poisoning male normal human porphyria reference value Spain
spellingShingle	Aminolevulinate dehydrase Porphobilinogen deaminase Reference range porphobilinogen deaminase porphobilinogen synthase adolescent adult aged article child cytosol enzyme activity enzyme analysis female heme synthesis human lead poisoning male normal human porphyria reference value Spain Santos, J.L. Fontanellas, A. Batlle, A.M.C. De Salamanca, R.E. Reference values of 5-Aminolevulinate dehydrase and Porphobilinogen deaminase in the Spanish population from Madrid
topic_facet	Aminolevulinate dehydrase Porphobilinogen deaminase Reference range porphobilinogen deaminase porphobilinogen synthase adolescent adult aged article child cytosol enzyme activity enzyme analysis female heme synthesis human lead poisoning male normal human porphyria reference value Spain
description	5-Aminolevulinate dehydrase (ALA-D) and porphobilinogen deaminase (PBG- D) are cytosolic enzymes involved in heme biosynthesis. ALA-D activity is altered both genetically and by the action of various environmental factors, including exposure to lead. The activity of PBG-D is reduced in acute intermittent porphyria. The aim of this work is to establish the 95% reference range of the erythrocytic activity of ALA-D and PBG-D in a control population. ALA-D activity limits were 15.8 and 50.2 nmol of PBG/ml of red blood cells (RBCS) per minute. For the activity of ALA-D restored by addition of zinc and dithiothreitol ('restored ALA-D'), these limits were 44.1 and 86.5 units. It has been found that the 'restored ALA-D'/ALA-D ratio is very useful for the evaluation of lead toxicity, and its 95% reference range was between 1.22 and 3.06. It has been demonstrated that the best method for measuring erythrocytic PBG-D is using PBG, but not ALA, as substrate; its 95% reference range was between 20.9 and 63.2 nmol of uroporphyrin/ml of RBCs per hour. Knowledge of these reference range values in a control population constitutes the basis for an accurate diagnosis of heavy metal intoxication and acute intermittent porphyria.
format	JOUR
author	Santos, J.L. Fontanellas, A. Batlle, A.M.C. De Salamanca, R.E.
author_facet	Santos, J.L. Fontanellas, A. Batlle, A.M.C. De Salamanca, R.E.
author_sort	Santos, J.L.
title	Reference values of 5-Aminolevulinate dehydrase and Porphobilinogen deaminase in the Spanish population from Madrid
title_short	Reference values of 5-Aminolevulinate dehydrase and Porphobilinogen deaminase in the Spanish population from Madrid
title_full	Reference values of 5-Aminolevulinate dehydrase and Porphobilinogen deaminase in the Spanish population from Madrid
title_fullStr	Reference values of 5-Aminolevulinate dehydrase and Porphobilinogen deaminase in the Spanish population from Madrid
title_full_unstemmed	Reference values of 5-Aminolevulinate dehydrase and Porphobilinogen deaminase in the Spanish population from Madrid
title_sort	reference values of 5-aminolevulinate dehydrase and porphobilinogen deaminase in the spanish population from madrid
url	http://hdl.handle.net/20.500.12110/paper_01476513_v39_n3_p168_Santos
work_keys_str_mv	AT santosjl referencevaluesof5aminolevulinatedehydraseandporphobilinogendeaminaseinthespanishpopulationfrommadrid AT fontanellasa referencevaluesof5aminolevulinatedehydraseandporphobilinogendeaminaseinthespanishpopulationfrommadrid AT batlleamc referencevaluesof5aminolevulinatedehydraseandporphobilinogendeaminaseinthespanishpopulationfrommadrid AT desalamancare referencevaluesof5aminolevulinatedehydraseandporphobilinogendeaminaseinthespanishpopulationfrommadrid
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Reference values of 5-Aminolevulinate dehydrase and Porphobilinogen deaminase in the Spanish population from Madrid

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