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Abstract:

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.

Registro:

Documento: Artículo
Título:Reference values of 5-Aminolevulinate dehydrase and Porphobilinogen deaminase in the Spanish population from Madrid
Autor:Santos, J.L.; Fontanellas, A.; Batlle, A.M.C.; De Salamanca, R.E.
Filiación:Porphyria Unit, Hosp. Universitario Doce de Octubre, Madrid, Spain
Ctro. de Invest. Sobre Porfirinas Y., Ciudad Universitaria, Pabellon II, 2do Piso, 1428 Buenos Aires, Argentina
Palabras clave: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
Año:1998
Volumen:39
Número:3
Página de inicio:168
Página de fin:171
DOI: http://dx.doi.org/10.1006/eesa.1997.1613
Título revista:Ecotoxicology and Environmental Safety
Título revista abreviado:Ecotoxicol. Environ. Saf.
ISSN:01476513
CODEN:EESAD
CAS:porphobilinogen deaminase, 9036-47-9, 9074-91-3; porphobilinogen synthase, 9036-37-7
Registro:https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_01476513_v39_n3_p168_Santos

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Citas:

---------- APA ----------
Santos, J.L., Fontanellas, A., Batlle, A.M.C. & De Salamanca, R.E. (1998) . Reference values of 5-Aminolevulinate dehydrase and Porphobilinogen deaminase in the Spanish population from Madrid. Ecotoxicology and Environmental Safety, 39(3), 168-171.
http://dx.doi.org/10.1006/eesa.1997.1613
---------- CHICAGO ----------
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" . Ecotoxicology and Environmental Safety 39, no. 3 (1998) : 168-171.
http://dx.doi.org/10.1006/eesa.1997.1613
---------- MLA ----------
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" . Ecotoxicology and Environmental Safety, vol. 39, no. 3, 1998, pp. 168-171.
http://dx.doi.org/10.1006/eesa.1997.1613
---------- VANCOUVER ----------
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. Ecotoxicol. Environ. Saf. 1998;39(3):168-171.
http://dx.doi.org/10.1006/eesa.1997.1613