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Abstract:

1. 1. Studies on porphyrin biosynthesis from exogenus ALA, at various time intervals as well as direct enzyme measurements (aminolevulimc acid dehydratase (ALA-D); porphobilinogenase (PBG ase) and deaminase were carried out in hemolysates of human erythrocytes from healthy controls and patients with lead poisoning (Pb), acute intermittent porphyria (AIP), porphyria cutanea tarda (PCT), erythropoietic protoporphyria (EPP), variegate porphyria (VP) and congenital erythropoietic porphyria (CEP). 2. 2. Inhibited ALA-D in Pb, reduced PBGase and deaminase in AIP, lower uroporphyrinogen decarboxylase in PCT, and diminished isomerase in CEP, were confirmed. In addition, ALA-D was found, reduced in AIP, unchanged in PCT and increased in EPP, VP and CEP. PBGase and deaminase were, on the other hand, increased in Pb and PCT, unchanged in VP and diminished in EPP and CEP. 3. 3. Total porphyrin biosynthesis is a function of time; compared to normals, is lower in CEP and AIP, but higher in PCT. 4. 4. The porphyrin profile changes along the time; uroporphyrin increases at longer intervals while that of coproporphyrin concomitantly diminished. A significance enhancement of octacarboxylic porphyrins was observed during the entire duration of the incubation in PCT hemolysates. In CEP the main porphyrin was always uroporphyrin I. 5. 5. Studies on both total porphyrins formed and their distribution were performed in hemolysates from cases of non-hereditary and hereditary PCT and AIP, before and after therapy. © 1980.

Registro:

Documento: Artículo
Título:Porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans
Autor:Parera, V.E.; Stella, A.M.; De Xifra, E.A.W.; Fukuda, H.; Del C. Batlle, A.M.
Filiación:Centro de Investigaciones sobre Porfirinas y Porfirias, CIPYP, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires y Consejo Nacional de Investigaciones Cientificas y Técnicas, CONICET, Ciudad Universitaria-Pabellón II 4 Piso, 1428 Buenos Aires, Argentina
Palabras clave:ammonia lyase; Ammonia Lyases; porphobilinogen deaminase; porphobilinogen synthase; porphyrin; acute disease; article; biosynthesis; blood; enzymology; erythrocyte; human; newborn; porphyria; reference value; skin disease; Acute Disease; Ammonia-Lyases; Erythrocytes; Human; Hydroxymethylbilane Synthase; Infant, Newborn; Porphobilinogen Synthase; Porphyria; Porphyrins; Reference Values; Skin Diseases; Support, Non-U.S. Gov't
Año:1980
Volumen:12
Número:5-6
Página de inicio:947
Página de fin:953
DOI: http://dx.doi.org/10.1016/0020-711X(80)90190-1
Título revista:International Journal of Biochemistry
Título revista abreviado:Int. J. Biochem.
ISSN:0020711X
CODEN:IJBOB
CAS:porphobilinogen deaminase, 9036-47-9, 9074-91-3; porphobilinogen synthase, 9036-37-7; porphyrin, 24869-67-8; Ammonia-Lyases, EC 4.3.1.; Hydroxymethylbilane Synthase, EC 4.3.1.8; Porphobilinogen Synthase, EC 4.2.1.24; Porphyrins
PDF:https://bibliotecadigital.exactas.uba.ar/download/paper/paper_0020711X_v12_n5-6_p947_Parera.pdf
Registro:https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_0020711X_v12_n5-6_p947_Parera

Referencias:

  • Batlle, Ferramola, Grinstein, Purification and general properties of ALA-D from cow liver (1967) Biochem. J., 104, pp. 244-249
  • Batlle A.M. Del, 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. 871-875
  • Batlle, Wider De Xifra, Stella, Bustos, With, Studies on porphyrin biosynthesis and the enzymes involved in bovine congenital erythropoietic porphyria (1979) Clin. Sci., 57, pp. 63-70
  • Batlle, Wider De Xifra, Stella, Bustos, Vazquez, Magnin, Porphyrin biosynthesis in normals and porphyries from precursors (1979) Revta argent. Derm.-Sif., 60, pp. 102-107
  • Doss, Metabolism of δ-aminolevulinic acid and porphobilinogen in human erythrocytes in acute intermittent porphyria (1973) Enzyme, 16, pp. 343-353
  • Hofstad, Seip, Eriksen, Congenital erythropoietic porphyria with a hitherto undescribed porphyrin pattern (1973) Acta Paediatrica, 26, pp. 380-384
  • Levin, Uropophyrinogen III cosynthetase in bovine erythropoietic porphyria (1968) Science, 161, pp. 907-908
  • Llambias, Batlle A.M. Del, Studies on the porphobilinogen deaminase-uroporphyrinogen cosynthetase system of cultured soybean cells (1971) Biochem. J., 107, pp. 327-340
  • Llambias, Batlle A.M. Del, Porphyrin biosynthesis VII-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
  • Miyagi, Petryka, Bossenmaier, Cardinal, Watson, The activities of uroporphyrinogen synthetase and cosynthetase in congenital erythropoietic porphyria (CEP) (1976) Am. J. Hemat., 1, pp. 3-21
  • Moore, Thompson, Goldberg, Ippen, Seubert, Seubert, The biosynthesis of haem in congenital erythropoietic porphyria (1978) Int. J. Biochem., 9, pp. 933-938
  • Piper, Tephly, Differential inhibition of erythrocyte and hepatic uroporphyrinogen synthetase activity by lead (1974) Life Sci., 14, pp. 873-876
  • Sancovich, Ferramola, Batlle, Kevilevich, Grinstein, Studies on cow liver porphobilinogen deaminase (1976) Acta physiol. latinoam., 26, pp. 379-385
  • Schermuly, Doss, Porphyrin biosynthesis from ALA and PBG by human erythrocytes in porphyrin disorders. Kinetic studies of the isomer series I and III (1976) Ann. clin. Res., 8, pp. 92-102
  • Tephly, Identification of a small molecular weight substance that protects Uroporphyrinogen I Synthetase from the inhibition by lead (1979) Porphyrins in Human Diseases—Report of Discussions, pp. 128-134. , M. Doss, P. Nawrocki, Falk, Freiburg
  • Viljoen, Becker, Kramer, A comparative study of porphyrin synthesis by whole blood and haemolysates from different mammalian species (1976) Comp. Biochem. Physiol., 55 B, pp. 151-155
  • Wider De Xifra, Batlle, Stella, Malamud, Acute Intermittent Porphyria—Another approach to therapy (1979) Int. J. Biochem., 12, pp. 819-822

Citas:

---------- APA ----------
Parera, V.E., Stella, A.M., De Xifra, E.A.W., Fukuda, H. & Del C. Batlle, A.M. (1980) . Porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans. International Journal of Biochemistry, 12(5-6), 947-953.
http://dx.doi.org/10.1016/0020-711X(80)90190-1
---------- CHICAGO ----------
Parera, V.E., Stella, A.M., De Xifra, E.A.W., Fukuda, H., Del C. Batlle, A.M. "Porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans" . International Journal of Biochemistry 12, no. 5-6 (1980) : 947-953.
http://dx.doi.org/10.1016/0020-711X(80)90190-1
---------- MLA ----------
Parera, V.E., Stella, A.M., De Xifra, E.A.W., Fukuda, H., Del C. Batlle, A.M. "Porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans" . International Journal of Biochemistry, vol. 12, no. 5-6, 1980, pp. 947-953.
http://dx.doi.org/10.1016/0020-711X(80)90190-1
---------- VANCOUVER ----------
Parera, V.E., Stella, A.M., De Xifra, E.A.W., Fukuda, H., Del C. Batlle, A.M. Porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans. Int. J. Biochem. 1980;12(5-6):947-953.
http://dx.doi.org/10.1016/0020-711X(80)90190-1