Enzyme replacement therapy in porphyrias. V. In vivo correction of δ-aminolaevulinate dehydratase defective in erythrocytes in lead intoxicated animals by enzyme-loaded red blood cell ghosts
Resealed erythrocyte ghosts are potential in vivo carriers of exogenous therapeutic enzymes. In the present work, the effect of administering δ-aminolaevulinate dehydratase (ALA-D) encapsulated in autologous red blood cell ghosts (RBCg) to lead intoxicated and normal mice was investigated. Administr...
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1989
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| Acceso en línea: | https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_08842884_v5_n2_p125_Bustos http://hdl.handle.net/20.500.12110/paper_08842884_v5_n2_p125_Bustos |
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paper:paper_08842884_v5_n2_p125_Bustos2023-06-08T15:46:33Z Enzyme replacement therapy in porphyrias. V. In vivo correction of δ-aminolaevulinate dehydratase defective in erythrocytes in lead intoxicated animals by enzyme-loaded red blood cell ghosts Aminolevulic acid dehydratase Enzyme replacement therapy Lead intoxication Porphyrias Red blood cell ghosts porphobilinogen synthase animal experiment animal model article autologous blood bioavailability blood level congenital disorder erythrocyte ghost head intoxication intravenous drug administration kidney liver mouse nonhuman porphyria priority journal spleen Animal Erythrocyte Membrane Erythrocytes Female Lead Poisoning Mice Mice, Inbred Strains Porphobilinogen Synthase Porphyria Support, Non-U.S. Gov't Resealed erythrocyte ghosts are potential in vivo carriers of exogenous therapeutic enzymes. In the present work, the effect of administering δ-aminolaevulinate dehydratase (ALA-D) encapsulated in autologous red blood cell ghosts (RBCg) to lead intoxicated and normal mice was investigated. Administration of ALA-D loaded RBCg to intoxicated mice produced an immediate and permanent recovery in erythrocytic ALA-D activity; the effect was also noticeable in liver and spleen and absent in kidney, indicating that the entrapped enzyme was significantly stabilized to allow retention of activity in both circulating and phagocytized cells. Administration of ALA-D loaded ghosts to normal mice did not produce significant changes in ALA-D activity in blood, liver, spleen or kidney. Administration of free exogenous enzyme or blood transfusion during lead intoxication was also ineffective; spontaneous recovery of the poisoned animals was very slow. We show that circulating as well as phagocytized ALA-D entrapped in autologous erythrocyte ghosts can be safe and beneficial in the treatment of lead poisoning. Clinical trial in patients is recommended. 1989 https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_08842884_v5_n2_p125_Bustos http://hdl.handle.net/20.500.12110/paper_08842884_v5_n2_p125_Bustos |
| institution |
Universidad de Buenos Aires |
| institution_str |
I-28 |
| repository_str |
R-134 |
| collection |
Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA) |
| topic |
Aminolevulic acid dehydratase Enzyme replacement therapy Lead intoxication Porphyrias Red blood cell ghosts porphobilinogen synthase animal experiment animal model article autologous blood bioavailability blood level congenital disorder erythrocyte ghost head intoxication intravenous drug administration kidney liver mouse nonhuman porphyria priority journal spleen Animal Erythrocyte Membrane Erythrocytes Female Lead Poisoning Mice Mice, Inbred Strains Porphobilinogen Synthase Porphyria Support, Non-U.S. Gov't |
| spellingShingle |
Aminolevulic acid dehydratase Enzyme replacement therapy Lead intoxication Porphyrias Red blood cell ghosts porphobilinogen synthase animal experiment animal model article autologous blood bioavailability blood level congenital disorder erythrocyte ghost head intoxication intravenous drug administration kidney liver mouse nonhuman porphyria priority journal spleen Animal Erythrocyte Membrane Erythrocytes Female Lead Poisoning Mice Mice, Inbred Strains Porphobilinogen Synthase Porphyria Support, Non-U.S. Gov't Enzyme replacement therapy in porphyrias. V. In vivo correction of δ-aminolaevulinate dehydratase defective in erythrocytes in lead intoxicated animals by enzyme-loaded red blood cell ghosts |
| topic_facet |
Aminolevulic acid dehydratase Enzyme replacement therapy Lead intoxication Porphyrias Red blood cell ghosts porphobilinogen synthase animal experiment animal model article autologous blood bioavailability blood level congenital disorder erythrocyte ghost head intoxication intravenous drug administration kidney liver mouse nonhuman porphyria priority journal spleen Animal Erythrocyte Membrane Erythrocytes Female Lead Poisoning Mice Mice, Inbred Strains Porphobilinogen Synthase Porphyria Support, Non-U.S. Gov't |
| description |
Resealed erythrocyte ghosts are potential in vivo carriers of exogenous therapeutic enzymes. In the present work, the effect of administering δ-aminolaevulinate dehydratase (ALA-D) encapsulated in autologous red blood cell ghosts (RBCg) to lead intoxicated and normal mice was investigated. Administration of ALA-D loaded RBCg to intoxicated mice produced an immediate and permanent recovery in erythrocytic ALA-D activity; the effect was also noticeable in liver and spleen and absent in kidney, indicating that the entrapped enzyme was significantly stabilized to allow retention of activity in both circulating and phagocytized cells. Administration of ALA-D loaded ghosts to normal mice did not produce significant changes in ALA-D activity in blood, liver, spleen or kidney. Administration of free exogenous enzyme or blood transfusion during lead intoxication was also ineffective; spontaneous recovery of the poisoned animals was very slow. We show that circulating as well as phagocytized ALA-D entrapped in autologous erythrocyte ghosts can be safe and beneficial in the treatment of lead poisoning. Clinical trial in patients is recommended. |
| title |
Enzyme replacement therapy in porphyrias. V. In vivo correction of δ-aminolaevulinate dehydratase defective in erythrocytes in lead intoxicated animals by enzyme-loaded red blood cell ghosts |
| title_short |
Enzyme replacement therapy in porphyrias. V. In vivo correction of δ-aminolaevulinate dehydratase defective in erythrocytes in lead intoxicated animals by enzyme-loaded red blood cell ghosts |
| title_full |
Enzyme replacement therapy in porphyrias. V. In vivo correction of δ-aminolaevulinate dehydratase defective in erythrocytes in lead intoxicated animals by enzyme-loaded red blood cell ghosts |
| title_fullStr |
Enzyme replacement therapy in porphyrias. V. In vivo correction of δ-aminolaevulinate dehydratase defective in erythrocytes in lead intoxicated animals by enzyme-loaded red blood cell ghosts |
| title_full_unstemmed |
Enzyme replacement therapy in porphyrias. V. In vivo correction of δ-aminolaevulinate dehydratase defective in erythrocytes in lead intoxicated animals by enzyme-loaded red blood cell ghosts |
| title_sort |
enzyme replacement therapy in porphyrias. v. in vivo correction of δ-aminolaevulinate dehydratase defective in erythrocytes in lead intoxicated animals by enzyme-loaded red blood cell ghosts |
| publishDate |
1989 |
| url |
https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_08842884_v5_n2_p125_Bustos http://hdl.handle.net/20.500.12110/paper_08842884_v5_n2_p125_Bustos |
| _version_ |
1768545515123769344 |