Porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans

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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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Autores principales: Parera, V.E., Stella, A.M., De Xifra, E.A.W., Fukuda, H., Del C. Batlle, A.M.
Formato: Artículo publishedVersion
Lenguaje:Inglés
Publicado: 1980
Materias:
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
Acceso en línea:http://hdl.handle.net/20.500.12110/paper_0020711X_v12_n5-6_p947_Parera
Aporte de:
Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA) de Universidad de Buenos Aires
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spelling paperaa:paper_0020711X_v12_n5-6_p947_Parera2023-06-12T16:42:10Z Porphyrin biosynthesis and enzymic studies in erythrocytes from normals and porphyric humans Int. J. Biochem. 1980;12(5-6):947-953 Parera, V.E. Stella, A.M. De Xifra, E.A.W. Fukuda, H. Del C. Batlle, A.M. 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 info:eu-repo/semantics/article info:ar-repo/semantics/artículo info:eu-repo/semantics/publishedVersion application/pdf eng info:eu-repo/semantics/openAccess http://creativecommons.org/licenses/by/2.5/ar 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)
language Inglés
orig_language_str_mv eng
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, 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
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.
format Artículo
Artículo
publishedVersion
author Parera, V.E.
Stella, A.M.
De Xifra, E.A.W.
Fukuda, H.
Del C. Batlle, A.M.
author_facet Parera, V.E.
Stella, A.M.
De Xifra, E.A.W.
Fukuda, H.
Del C. Batlle, A.M.
author_sort Parera, V.E.
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 http://hdl.handle.net/20.500.12110/paper_0020711X_v12_n5-6_p947_Parera
work_keys_str_mv AT parerave porphyrinbiosynthesisandenzymicstudiesinerythrocytesfromnormalsandporphyrichumans
AT stellaam porphyrinbiosynthesisandenzymicstudiesinerythrocytesfromnormalsandporphyrichumans
AT dexifraeaw porphyrinbiosynthesisandenzymicstudiesinerythrocytesfromnormalsandporphyrichumans
AT fukudah porphyrinbiosynthesisandenzymicstudiesinerythrocytesfromnormalsandporphyrichumans
AT delcbatlleam porphyrinbiosynthesisandenzymicstudiesinerythrocytesfromnormalsandporphyrichumans
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