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Abstract:

1. 1. This paper confirms the increase in sensitivity obtained for erythrocyte UIS measurement by pre-incubation of the red cells in a 0.2% Triton-X 100 solution containing 1 mmol/1 ZnSO4 and dithiothreitol as described by Piepkorn et al. (1978). 2. 2. To achieve optimal precision in this assay a substrate concentration of delta aminolaevulinic acid (ALA) of 1000nmol/ml is required. Interpretation of the results obtained by this method is discussed and its use in the detection of both latent and acute intermittent porphyria is demonstrated. Comparative studies were carried out by using the Batlle et al. (1978) method and a modification employing ALA as substrate. © 1980.

Registro:

Documento: Artículo
Título:The measurement of erythrocyte uroporphyrinogen i synthase in the diagnosis of latent and acute intermittent porphyria
Autor:Brocklehurst, D.; Wider De Xifra, E.A.; Batlle, A.
Filiación:Area Department of Clinical Chemistry, Doncaster Royal Infirmary, Doncaster, England, United Kingdom
Centro de Investigaciones sobre Porfirinas y Porfirias (CIPYP), Facultad de Ciencias Exactas y Naturales, University of Buenos Aires, Pabellon II-4 to Piso-Nunez, 1428 Buenos Aires, Argentina
Palabras clave:blood and hemopoietic system; erythrocyte; methodology; porphyria; uroporphyrinogen synthase; Acute Disease; Adult; Aged; Ammonia-Lyases; Chronic Disease; Comparative Study; Enzyme Tests; Erythrocytes; Female; Human; Hydroxymethylbilane Synthase; Kinetics; Male; Pedigree; Porphyria
Año:1980
Volumen:12
Número:5-6
Página de inicio:791
Página de fin:793
DOI: http://dx.doi.org/10.1016/0020-711X(80)90164-0
Título revista:International Journal of Biochemistry
Título revista abreviado:Int. J. Biochem.
ISSN:0020711X
CODEN:IJBOB
CAS:Ammonia-Lyases, EC 4.3.1.; Hydroxymethylbilane Synthase, EC 4.3.1.8
PDF:https://bibliotecadigital.exactas.uba.ar/download/paper/paper_0020711X_v12_n5-6_p791_Brocklehurst.pdf
Registro:https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_0020711X_v12_n5-6_p791_Brocklehurst

Referencias:

  • Astrup, Family studies on the activity of uroporphyrinogen I synthase in diagnosis of acute intermittent porphyria (1978) Clin. Sci. Mol. Med., 54, pp. 251-256
  • del Batlle, Wider De Xifra, Stella, A simple method for measuring erythrocyte porphobilinogenase and its use in the diagnosis of Acute Intermittent Porphyria (1978) Int. J. Biochem., 9, pp. 871-876
  • Wider De Xifra, del Batlle, Stella, Malamud, Acute intermittent porphyria—another approach to therapy (1980) Int. J. Biochem, 12, pp. 819-822
  • Granick, Sassa, Granick, Levere, Kappas, Assays for porphyrins, delta aminolae-vulinic acid dehydratase and porphyrinogen synthetase in microlitre samples of whole blood: applications to metabolic defects involving the heme pathway (1972) Proc. natn. Acad. Sci. U.S.A., 69, pp. 2381-2385
  • Peterson, Hamernyik, Bird, Labbe, Erythrocyte uroporphyrinogen I synthase activity in diagnosis of acute intermittent porphyria (1976) Clin. Chem., 22, pp. 1835-1840
  • Piepkorn, Hamernyik, Labbe, A modified erythrocyte uroporphyrinogen I synthase assay and its clinical interpretation (1978) Clin. Chem., 24, pp. 1751-1755

Citas:

---------- APA ----------
Brocklehurst, D., Wider De Xifra, E.A. & Batlle, A. (1980) . The measurement of erythrocyte uroporphyrinogen i synthase in the diagnosis of latent and acute intermittent porphyria. International Journal of Biochemistry, 12(5-6), 791-793.
http://dx.doi.org/10.1016/0020-711X(80)90164-0
---------- CHICAGO ----------
Brocklehurst, D., Wider De Xifra, E.A., Batlle, A. "The measurement of erythrocyte uroporphyrinogen i synthase in the diagnosis of latent and acute intermittent porphyria" . International Journal of Biochemistry 12, no. 5-6 (1980) : 791-793.
http://dx.doi.org/10.1016/0020-711X(80)90164-0
---------- MLA ----------
Brocklehurst, D., Wider De Xifra, E.A., Batlle, A. "The measurement of erythrocyte uroporphyrinogen i synthase in the diagnosis of latent and acute intermittent porphyria" . International Journal of Biochemistry, vol. 12, no. 5-6, 1980, pp. 791-793.
http://dx.doi.org/10.1016/0020-711X(80)90164-0
---------- VANCOUVER ----------
Brocklehurst, D., Wider De Xifra, E.A., Batlle, A. The measurement of erythrocyte uroporphyrinogen i synthase in the diagnosis of latent and acute intermittent porphyria. Int. J. Biochem. 1980;12(5-6):791-793.
http://dx.doi.org/10.1016/0020-711X(80)90164-0