A comparative study of porphyrin synthesis by whole blood and haemolysates from different mammalian species

Viljoen, J. D.; Becker, David M.; Kramer, S. D.

doi:10.1016/0305-0491(76)90189-9

Comparative Biochemistry and Physiology Part B: Comparative Bio

1976

DOI: 10.1016/0305-0491(76)90189-9

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A comparative study of porphyrin synthesis by whole blood and haemolysates from different mammalian species

J. D. Viljoen

David M. Becker

S. D. Kramer

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1980

1986

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Cited by 2 publications

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“…It has been already observed by others and in our laboratory (Doss, 1973;Schermuly & Doss, 1976;Viljoen et al, 1976;Miyagi et al, 1976;Batlle et al, 1979a,b), that information about the underlying enzymic defect and inheritance in many porphyrias can also be obtained by carrying out in oitro studies on porphyrin biosynthesis from ALA and PBG by erythrocytes, under different experimental conditions as well as by direct enzyme measurements.…”

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confidence: 60%

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

Parera

Stella

Xifra

et al. 1980

International Journal of Biochemistry

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Studies on porphyrin biosynthesis from exogenus ALA, at various time intervals as well as direct enzyme measurements (aminolevulinic 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. 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 PC?. unchanged in VP and diminished in EPP and CEP.3. Total porphyrin biosynthesis is a function of time; compared to normals, is lower in CEP and AIP, but higher in PCT.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. 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.

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mentioning

confidence: 60%

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

Parera

Stella

Xifra

et al. 1980

International Journal of Biochemistry

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Inhibition of the biosynthesis of uroporphyrinogen and heme in rat liver during obstructive jaundice produced by bile duct ligation

Piper

Tse

Sadler

et al. 1986

Archives of Biochemistry and Biophysics

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A comparative study of porphyrin synthesis by whole blood and haemolysates from different mammalian species

Cited by 2 publications

References 5 publications

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

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

Inhibition of the biosynthesis of uroporphyrinogen and heme in rat liver during obstructive jaundice produced by bile duct ligation

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