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Abstract:

1. 1. Resealed erythrocytes ghosts are potential in vivo carriers of exogenous therapeutic enzymes. The present work is part of studies directed toward the use of enzymes encapsulated within artificial or natural cellular carriers, in enzyme replacement therapy in porphyrias. 2. 2. Lead intoxication was chosen as a model system for these experiments. 3. 3. The optimum conditions for both the preparation of erythrocyte ghosts and encapsulation of δ-aminolaevulinate dehydratase (ALA-D) were studied in detail and a very good yield procedure for entrapment of exogenous highly purified human blood ALA-D, into both normal and defective erythrocytes was developed. 4. 4. The capacity of ALA-D defective cells of lead intoxicated patients and animals was brought within the normal range, in vitro, either by introducing ALA-D into them or by mixing with ALA-D loaded erythrocyte ghosts. 5. 5. Red blood cell ghosts entrapment is therefore a promising approach to the delivery of ALA-D when applying enzyme replacement therapy in lead poisoning. © 1983.

Registro:

Documento: Artículo
Título:Enzyme replacement therapy in porphyrias-III: Potential use of erythrocyte ghosts as carriers of δ-aminolaevulinate dehydratase
Autor:Bustos, N.L.; Stella, A.M.; Wider, E.A.; Del C. Batlle, A.M.
Filiación:Centro de Investigaciones sobre Porfirinas y Porfirias, CIPYP (CONICET and FCE y N, University of Buenos Aires), Ciudad Universitaria, Pab. II, 1428 Búenos Aires, Argentina
Palabras clave:porphobilinogen synthase; animal cell; blood and hemopoietic system; drug blood level; drug delivery system; drug efficacy; enzyme replacement; enzyme therapy; erythrocyte ghost; human; human cell; intoxication; porphyria; therapy
Año:1983
Volumen:15
Número:3
Página de inicio:447
Página de fin:452
DOI: http://dx.doi.org/10.1016/0020-711X(83)90116-7
Título revista:International Journal of Biochemistry
Título revista abreviado:Int. J. Biochem.
ISSN:0020711X
CODEN:IJBOB
CAS:porphobilinogen synthase, 9036-37-7
Registro:https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_0020711X_v15_n3_p447_Bustos

Referencias:

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  • Batlle A.M. del, Grinstein, Porphyrin biosynthesis studies on erythrocyte preparations (1964) Biochimica et Biophysica Acta (BBA) - General Subjects, 82, pp. 1-10
  • Batlle A.M. del, Ferramola, Grinstein, Purification and general properties of Delta Aminolaevulic Acid Dehydratase from cow liver (1967) Biochem. J., 104, pp. 244-249
  • Batlle A.M. del, Wider de Xifra, Stella, Bustos, With, Studies on porphyrin biosynthesis and the enzymes involved in bovine eongenital erythropoietie porphyria (1979) Clin. Sci., 57, pp. 63-70
  • Bustos, Stella, Wider de xifra, Batlle A.M. del, Studies on erythrocyte aminolaevulinate dehydratase I. Its purification and possible therapeutic applications (1980) Int. J. Biochem., 12, pp. 745-749
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  • Espinola, Wider, Stella, del Batlle, Enzyme replacement therapy in Porphyrias II: Entrapment of δ-aminolaevulinate dehydratase in liposomes (1983) Int. J. Biochem., 15, pp. 439-445
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  • Seeman, Transient holes in the erythrocyte membrane during hypotonie hemolysis and stable holes in the membrane after lysis by saponin and lysolecithin (1967) J. cell. Biol., 32, pp. 55-70
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Citas:

---------- APA ----------
Bustos, N.L., Stella, A.M., Wider, E.A. & Del C. Batlle, A.M. (1983) . Enzyme replacement therapy in porphyrias-III: Potential use of erythrocyte ghosts as carriers of δ-aminolaevulinate dehydratase. International Journal of Biochemistry, 15(3), 447-452.
http://dx.doi.org/10.1016/0020-711X(83)90116-7
---------- CHICAGO ----------
Bustos, N.L., Stella, A.M., Wider, E.A., Del C. Batlle, A.M. "Enzyme replacement therapy in porphyrias-III: Potential use of erythrocyte ghosts as carriers of δ-aminolaevulinate dehydratase" . International Journal of Biochemistry 15, no. 3 (1983) : 447-452.
http://dx.doi.org/10.1016/0020-711X(83)90116-7
---------- MLA ----------
Bustos, N.L., Stella, A.M., Wider, E.A., Del C. Batlle, A.M. "Enzyme replacement therapy in porphyrias-III: Potential use of erythrocyte ghosts as carriers of δ-aminolaevulinate dehydratase" . International Journal of Biochemistry, vol. 15, no. 3, 1983, pp. 447-452.
http://dx.doi.org/10.1016/0020-711X(83)90116-7
---------- VANCOUVER ----------
Bustos, N.L., Stella, A.M., Wider, E.A., Del C. Batlle, A.M. Enzyme replacement therapy in porphyrias-III: Potential use of erythrocyte ghosts as carriers of δ-aminolaevulinate dehydratase. Int. J. Biochem. 1983;15(3):447-452.
http://dx.doi.org/10.1016/0020-711X(83)90116-7