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Literature DB >> 6615434

Simultaneous determination of hydroxymethylbilane synthase and uroporphyrinogen III synthase in erythrocytes by high-performance liquid chromatography.

Abstract

A high-performance-liquid-chromatographic method is developed for the simultaneous determination of hydroxymethylbilane synthase and uroporphyrinogen III synthase activity in erythrocytes. Effective separation of uroporphyrin I and III isomers allows the accurate quantification of individual isomers and the total uroporphyrin concentration. Total uroporphyrin production is used to calculate hydroxymethylbilane synthase activity, and the amount of uroporphyrin III formed represents the activity of uroporphyrinogen III synthase. Normal ranges are established for the two enzymes.

Entities: Chemical Gene

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Year: 1983 PMID： 6615434 PMCID： PMC1152093 DOI： 10.1042/bj2130085

Source DB: PubMed Journal: Biochem J ISSN： 0264-6021 Impact factor: 3.857

10 in total

Review 1. Comparative aspects of porphyria in man and animals.

Authors: E Y Levin
Journal: Ann N Y Acad Sci Date: 1975-04-15 Impact factor: 5.691

2. A simple method for measuring erythrocyte porphobilinogenase, and its use in the diagnosis of acute intermittent porphyria.

Authors: A M Batlle; E A Wider de Xifra; A M Stella
Journal: Int J Biochem Date: 1978

3. Macrocyclic intermediates in the biosynthesis of porphyrins.

Authors: A H Jackson; H A Sancovich; A M Ferramola; N Evans; D E Games; S A Matlin; G H Elder; S G Smith
Journal: Philos Trans R Soc Lond B Biol Sci Date: 1976-02-05 Impact factor: 6.237

4. A red cell enzyme method for the diagnosis of acute intermittent porphyria.

Authors: C R Magnussen; J B Levine; J M Doherty; J O Cheesman; D P Tschudy
Journal: Blood Date: 1974-12 Impact factor: 22.113

5. Enzymatic properties of uroporphyrinogen 3 cosynthetase.

Authors: E Y Levin
Journal: Biochemistry Date: 1971-12-07 Impact factor: 3.162

6. Uroporphyrinogen 3 cosynthetase activity in fibroblasts from patients with congenital erythropoietic porphyria.

Authors: G Romeo; M M Kaback; E Y Levin
Journal: Biochem Genet Date: 1970-12 Impact factor: 1.890

7. A rapid direct assay for uroporphyrinogen III cosynthetase.

Authors: P M Jordan; H Nordlov; G Burton; A I Scott
Journal: FEBS Lett Date: 1980-06-30 Impact factor: 4.124

8. Decreased red cell uroporphyrinogen I synthetase activity in intermittent acute porphyria.

Authors: L J Strand; U A Meyer; B F Felsher; A G Redeker; H S Marver
Journal: J Clin Invest Date: 1972-10 Impact factor: 14.808

9. Heme biosynthesis in intermittent acute prophyria: decreased hepatic conversion of porphobilinogen to porphyrins and increased delta aminolevulinic acid synthetase activity.

Authors: L J Strand; B F Felsher; A G Redeker; H S Marver
Journal: Proc Natl Acad Sci U S A Date: 1970-11 Impact factor: 11.205

10. Congenital erythropoietic porphyria (Günther's disease): enzymatic studies on two cases of late onset.

Authors: J C Deybach; H de Verneuil; N Phung; Y Nordmann; A Puissant; B Boffety
Journal: J Lab Clin Med Date: 1981-04

10 in total

5 in total

1. Increased excretion of coproporphyrin I in a patient with hereditary tyrosinaemia type I: relevant changes with NTBC treatment.

Authors: C Depetris-Boldini; R Galetto; M P Videla; R de Kremer Dodelson
Journal: J Inherit Metab Dis Date: 1999-05 Impact factor: 4.982

2. Congenital erythropoietic porphyria due to a mutation in GATA1: the first trans-acting mutation causative for a human porphyria.

Authors: John D Phillips; David P Steensma; Michael A Pulsipher; Gerald J Spangrude; James P Kushner
Journal: Blood Date: 2006-12-05 Impact factor: 22.113