PMID: 4067519Sep 1, 1985

"Glucose effect" and rate limiting function of uroporphyrinogen synthase on porphyrin metabolism in hepatocyte culture: relationship with human acute hepatic porphyrias

Journal of Clinical Chemistry and Clinical Biochemistry. Zeitschrift Für Klinische Chemie Und Klinische Biochemie
M DossF Verspohl

Abstract

The effect of glucose on drug-promoted induction of porphyrin synthesis was studied in chick embryo liver cell culture with and without the addition of exogenous delta-aminolaevulinic acid (ALA). Induction of ALA synthase was abolished by haemin or glucose. Less than 10% of porphobilinogen is converted into protoporphyrin. Protoporphyrin synthesis cannot be enhanced by high ALA concentrations. The conversion of exogenous ALA into porphyrins decreases with increasing concentrations of ALA from 0.1 to 2.0 mmol/l, whereas porphobilinogen accumulates, thus reflecting the rate limiting function of uroporphyrinogen synthase, which is not influenced by glucose. This needle-eye-like function of uroporphyrinogen synthase within the porphyrin biosynthetic chain explains the urinary increase of ALA and porphobilinogen in the acute phase of variegate and coproporphyria, similar to that in acute intermittent porphyria. The "glucose effect" was also investigated in vivo in humans in 32 courses of hereditary acute hepatic porphyrias (acute intermittent porphyria, variegate porphyria, coproporphyria and porphobilinogen synthase defect porphyria). Patients were treated with high carbohydrate intake (approximately 500 g/24 h), mainly in the form...Continue Reading

References

Sep 12, 1979·Biochemical and Biophysical Research Communications·G SrivastavaW H Elliott
Aug 17, 1967·The New England Journal of Medicine·K B KnudsenF Lecocq
Jun 15, 1972·The New England Journal of Medicine·U A MeyerH S Marver
Apr 11, 1968·The New England Journal of Medicine·P Koskelo, R Pelkonen
May 1, 1982·Seminars in Liver Disease·H L Bonkowsky, W Schady
Jul 1, 1981·Klinische Wochenschrift·M Doss, F Verspohl
Aug 15, 1980·Klinische Wochenschrift·C A PierachC J Watson

Citations

Mar 9, 2004·Toxicology·María Cristina TairaLeonor Carmen San Martín de Viale
Mar 29, 2003·Journal of Hepatology·Thomas S WeissWolfgang E Thasler
Jul 5, 2011·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·Neil G Simon, Geoffrey K Herkes
Nov 13, 2007·Biochemical Pharmacology·Sandra Marcela LelliL San Martín de Viale

Related Concepts

Aminolevulinic Acid Hydrochloride
5-Aminolevulinate synthase
Ammonia-Lyases
Chick Embryo
Glucose, (beta-D)-Isomer
Heme
Liver
Liver Dysfunction
Meprospan
Porphobilinogen

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