Literature DB >> 14605502

A description of an HPLC assay of coproporphyrinogen III oxidase activity in mononuclear cells.

U Gross1, R Gerlach, A Kühnel, V Seifert, M O Doss.   

Abstract

Coproporphyrinogen III oxidase is deficient in hereditary coproporphyria. An activity assay for this enzyme in mononuclear cells, besides the preparation of the substrate, are presented. The separation conditions for the product of the test protoporphyrin IX by gradient, reversed-phase high-performance liquid chromatography are given. The normal value from mononuclear cells of healthy volunteers was 138 +/- 21 pkat/g total soluble protein (mean +/- SD). The enzyme activity of a family with hereditary coproporphyria was measured. The gene carriers exhibit a specific coproporphyrinogen III oxidase activity of 61-90 pkat/g total soluble protein.

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Year:  2003        PMID： 14605502     DOI： 10.1023/a:1025952031660

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


  12 in total

1.  Compound heterozygous hereditary coproporphyria with fluorescing teeth.

Authors:  M O Doss; U Gross; J Lamoril; C Kranl; K Jacob; M Doss; V da Silva; A G Freesemann; J C Deybach; N Sepp; Y Nordmann
Journal:  Ann Clin Biochem       Date:  1999-09       Impact factor: 2.057

2.  Neonatal-onset hereditary coproporphyria with male pseudohermaphrodism.

Authors:  H Takeuchi; M Kondo; M Daimon; S Susa; K Ueoka; O Uemura; H Togari
Journal:  Blood       Date:  2001-12-15       Impact factor: 22.113

Review 3.  Hereditary coproporphyria.

Authors:  P Martásek
Journal:  Semin Liver Dis       Date:  1998       Impact factor: 6.115

4.  DGGE analysis of the coproporphyrinogen oxidase gene: two new mutations in DNA from Danish patients with hereditary coproporphyria.

Authors:  N E Petersen; M Käehne; L Christiansen; A Brock; O Hother-Nielsen; K Rasmussen
Journal:  Scand J Clin Lab Invest       Date:  2000-11       Impact factor: 1.713

5.  Separation of lymphocytes, granulocytes, and monocytes from human blood using iodinated density gradient media.

Authors:  A Bøyum
Journal:  Methods Enzymol       Date:  1984       Impact factor: 1.600

Review 6.  Molecular, immunological, enzymatic and biochemical studies of coproporphyrinogen oxidase deficiency in a family with hereditary coproporphyria.

Authors:  U Gross; H Puy; A Kühnel; U Meissauer; J C Deybach; K Jacob; P Martasek; Y Nordmann; M O Doss
Journal:  Cell Mol Biol (Noisy-le-grand)       Date:  2002-02       Impact factor: 1.770

7.  Hereditary coproporphyria in Germany: clinical-biochemical studies in 53 patients.

Authors:  A Kühnel; U Gross; M O Doss
Journal:  Clin Biochem       Date:  2000-08       Impact factor: 3.281

8.  A molecular, enzymatic and clinical study in a family with hereditary coproporphyria.

Authors:  U Gross; H Puy; U Meissauer; J Lamoril; J C Deybach; M Doss; Y Nordmann; M O Doss
Journal:  J Inherit Metab Dis       Date:  2002-08       Impact factor: 4.982

9.  Oxygen-independent coproporphyrinogen-III oxidase HemN from Escherichia coli.

Authors:  Gunhild Layer; Knut Verfürth; Esther Mahlitz; Dieter Jahn
Journal:  J Biol Chem       Date:  2002-07-11       Impact factor: 5.157

10.  Accurate and specific HPLC assay of coproporphyrinogen III oxidase activity in human peripheral leucocytes.

Authors:  R Guo; C K Lim; T J Peters
Journal:  Clin Chim Acta       Date:  1988-10-31       Impact factor: 3.786
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  1 in total

1.  Direct assay of enzymes in heme biosynthesis for the detection of porphyrias by tandem mass spectrometry. Uroporphyrinogen decarboxylase and coproporphyrinogen III oxidase.

Authors:  Yuesong Wang; Paula Gatti; Martin Sadílek; C Ronald Scott; Frantisek Turecek; Michael H Gelb
Journal:  Anal Chem       Date:  2008-02-23       Impact factor: 6.986
  1 in total

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