Porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans
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...
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1980
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| Acceso en línea: | https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_0020711X_v12_n5-6_p947_Parera http://hdl.handle.net/20.500.12110/paper_0020711X_v12_n5-6_p947_Parera |
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paper:paper_0020711X_v12_n5-6_p947_Parera2023-06-08T14:41:05Z Porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans Parera, Victoria Estela Stella de Rosellini, Ana María Cristina Fukuda, Haydeé Batlle, Alcira María del Carmen 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 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. Fil:Parera, V.E. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Stella, A.M. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Fukuda, H. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Del C. Batlle, A.M. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. 1980 https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_0020711X_v12_n5-6_p947_Parera http://hdl.handle.net/20.500.12110/paper_0020711X_v12_n5-6_p947_Parera |
| institution |
Universidad de Buenos Aires |
| institution_str |
I-28 |
| repository_str |
R-134 |
| collection |
Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA) |
| topic |
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 |
| spellingShingle |
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 Parera, Victoria Estela Stella de Rosellini, Ana María Cristina Fukuda, Haydeé Batlle, Alcira María del Carmen Porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans |
| topic_facet |
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 |
| description |
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. |
| author |
Parera, Victoria Estela Stella de Rosellini, Ana María Cristina Fukuda, Haydeé Batlle, Alcira María del Carmen |
| author_facet |
Parera, Victoria Estela Stella de Rosellini, Ana María Cristina Fukuda, Haydeé Batlle, Alcira María del Carmen |
| author_sort |
Parera, Victoria Estela |
| title |
Porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans |
| title_short |
Porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans |
| title_full |
Porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans |
| title_fullStr |
Porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans |
| title_full_unstemmed |
Porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans |
| title_sort |
porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans |
| publishDate |
1980 |
| url |
https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_0020711X_v12_n5-6_p947_Parera http://hdl.handle.net/20.500.12110/paper_0020711X_v12_n5-6_p947_Parera |
| work_keys_str_mv |
AT pareravictoriaestela porphyrinbiosynthesisandenzymicstudiesinerythrocytesfromnormalsandporphyrichumans AT stelladerosellinianamariacristina porphyrinbiosynthesisandenzymicstudiesinerythrocytesfromnormalsandporphyrichumans AT fukudahaydee porphyrinbiosynthesisandenzymicstudiesinerythrocytesfromnormalsandporphyrichumans AT batllealciramariadelcarmen porphyrinbiosynthesisandenzymicstudiesinerythrocytesfromnormalsandporphyrichumans |
| _version_ |
1768542923467522048 |