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Abstract:

1. l. A simple method for measuring erythrocyte porphobilinogenase activity has been devised. 2. 2. Red blood cells are hemolysed by three freeze-thaws, the hemolysate is diluted 15 times and only 0.5 ml of this preparation is used for determining porphobilinogenase. in an incubation system also containing porphobilinogen, NaCl. MgCl2. Tris-HCl buffer pH 8.2; which is incubated aerobically, in the dark. at 45°. for 1 hr with shaking. 3. 3. The enzymic activity is quenched by adding TCA. 4. 4. Porphyrinogens are oxidized, protein precipitated centrifuged off and uroporphyrins formed are determined spectrophotometrically in the supernatant. 5. 5. The enzyme is stable up to 1 month if stored at -20°. © 1978.

Registro:

Documento: Artículo
Título:A simple method for measuring erythrocyte porphobilinogenase, and its use in the diagnosis of acute intermittent porphyria
Autor:Del C. Batlle, A.M.; De Xifra, E.A.W.; MarÍa Stella, A.
Filiación:Centro de Investigaciones sobre Porfirinas, Porfirias (CIPYP). Departamento, Química Biológica. Facultad de Ciencias Exactas y Naturales, Ciudad Universitaria, Muriez, 1428 Buenos Aires, Argentina
Palabras clave:erythrocyte enzyme; acute intermittent porphyria; blood and hemopoietic system; diagnosis; methodology; porphobilinogenase; Aminolevulinic Acid; Ammonia-Lyases; Comparative Study; Erythrocytes; Hemolysis; Human; Hydrogen-Ion Concentration; Porphobilinogen; Porphyria; Spectrophotometry
Año:1978
Volumen:9
Número:12
Página de inicio:871
Página de fin:875
DOI: http://dx.doi.org/10.1016/0020-711X(78)90062-9
Título revista:International Journal of Biochemistry
Título revista abreviado:Int. J. Biochem.
ISSN:0020711X
CODEN:IJBOB
CAS:Aminolevulinic Acid, 106-60-5; Ammonia-Lyases, EC 4.3.1.; Porphobilinogen, 487-90-1
Registro:https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_0020711X_v9_n12_p871_DelCBatlle

Referencias:

  • Batlle, Del, Biochemical research of the porphyrin and haem unit at the department of biochemistry, school of sciences (1973) Int. J. Biochem., 4, pp. 425-436. , University of Buenos Aires, Argentina
  • Batlle, Del, Grinstein, Porphyrin biosynthesis I. Studies on erythrocyte preparations (1964) Biochimica et Biophysica Acta (BBA) - General Subjects, 82, pp. 1-12
  • Batlle, Del, Rossetti, Enzymic polymerization of porphobilinogen into uroporphyr-inogens. (Review.) (1977) Int. J. Biochem., 8, pp. 259-267
  • Granick, Sassa, Granick, Levere, Kappas, Assays for porphyrins. δ-aminolevu-linic acid dehydratase and porphyrinogen synthetase in microliter samples of whole blood: applications to metabolic defects involving the heme pathway (1972) Proc. natn Acad. Sci., U.S.A., 69, pp. 2381-2386
  • Kappas, Sassa, Granick, Bradlow, Endocrine-gene interaction in the pathogenesis of acute intermittent porphyria (1974) Brain Dysfunction in Metabolic Disorders, 53, pp. 225-237. , Plunf. Res. Publ. Assoc. Nerv. Ment. Dis. Raven Press, New York
  • Lockwood, Rimington, Purification of an enzyme converting porphobilinogen to uroporphyrin (1957) Biochem. J., 67, p. 8
  • Llambias, Batlle, Del, Studies on the porphobilinogen deaminase-uroporphyrinogen cosynthetase system of cultured soya-bean cells (1971) Biochem. J., 107, pp. 327-340
  • Llambias, Batlle, Del, Porphyrin biosynthesis. VIII. Avian erythrocyte porphobilinogen deaminase-uroporphyrinogen III cosynthetase, its purification, properties and the separation of its components (1971) Biochimica et Biophysica Acta (BBA) - Enzymology, 227, pp. 180-191
  • Magnussen, Levine, Doherty, Cheesman, Tschudy, A red cell enzyme method for the diagnosis of acute intermittent porphyria (1974) Blood, 44, pp. 857-868
  • Mauzerall, Granick, Occurrence and determination of δ-aminolevulinic acid and porphobilinogen in urine (1956) J. biol. Chem., 219, pp. 435-446
  • Meyer, Intermittent acute porphyria- clinical and biochemical studies of disordered heme biosynthesis (1973) Enzyme, 16, pp. 334-342
  • Sancovich, Ferramola, Batele, Dee, Grinstein, Preparation of porphobilinogen (1970) Methods in Enzymology, 17, p. 220. , H. Tabor, C.W. Tabor, Academic Press, New York
  • Steela, Batele, Del, Rate of concentration and digestion of radioactive growth hormone preparations injected in rats, as measured by the amount and nature of radioactivity in the tissues (1977) Molecular and Cellular Biochemistry, 16, pp. 87-104
  • Strand, Meyer, Felsher, Redecker, Marver, Decreased red cell uroporphyrinogen I synthetase activity in acute intermittent porphyria (1972) J. clin. Inrest., 51, pp. 2530-2535

Citas:

---------- APA ----------
Del C. Batlle, A.M., De Xifra, E.A.W. & MarÍa Stella, A. (1978) . A simple method for measuring erythrocyte porphobilinogenase, and its use in the diagnosis of acute intermittent porphyria. International Journal of Biochemistry, 9(12), 871-875.
http://dx.doi.org/10.1016/0020-711X(78)90062-9
---------- CHICAGO ----------
Del C. Batlle, A.M., De Xifra, E.A.W., MarÍa Stella, A. "A simple method for measuring erythrocyte porphobilinogenase, and its use in the diagnosis of acute intermittent porphyria" . International Journal of Biochemistry 9, no. 12 (1978) : 871-875.
http://dx.doi.org/10.1016/0020-711X(78)90062-9
---------- MLA ----------
Del C. Batlle, A.M., De Xifra, E.A.W., MarÍa Stella, A. "A simple method for measuring erythrocyte porphobilinogenase, and its use in the diagnosis of acute intermittent porphyria" . International Journal of Biochemistry, vol. 9, no. 12, 1978, pp. 871-875.
http://dx.doi.org/10.1016/0020-711X(78)90062-9
---------- VANCOUVER ----------
Del C. Batlle, A.M., De Xifra, E.A.W., MarÍa Stella, A. A simple method for measuring erythrocyte porphobilinogenase, and its use in the diagnosis of acute intermittent porphyria. Int. J. Biochem. 1978;9(12):871-875.
http://dx.doi.org/10.1016/0020-711X(78)90062-9