Literature DB >> 947611

The spectrophotometric determination of uroporphyrinogen I synthetase activity.

B Grandchamp, N Phung, M Grelier, Y Nordmann.   

Abstract

A simple spectrophotometric method for uroporphyrinogen I synthetase in erythrocytes is described. Results obtained on intermittent acute porphyria patients and carriers are similar to the results obtained with fluorimetric methods. Reproducibility, relationship between enzyme activity and enzyme concentration, and effect of time on enzymatic activity are described.

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Year:  1976        PMID： 947611     DOI： 10.1016/0009-8981(76)90011-5

Source DB:  PubMed          Journal:  Clin Chim Acta        ISSN： 0009-8981            Impact factor:   3.786


  10 in total

1.  Aucte intermittent porphyria and epilepsy.

Authors:  R Biagini; R Tignani; A R Fifi; L Nappini
Journal:  Arch Dis Child       Date:  1979-08       Impact factor: 3.791

2.  Pro-oxidant and antioxidant factors in acute intermittent porphyria: family studies.

Authors:  E Rocchi; P Ventura; A Ronzoni; M C Rosa; C Gozzi; L Marri; G Casalgrandi; M D Cappellini
Journal:  J Inherit Metab Dis       Date:  2004       Impact factor: 4.982

3.  A point mutation G----A in exon 12 of the porphobilinogen deaminase gene results in exon skipping and is responsible for acute intermittent porphyria.

Authors:  B Grandchamp; C Picat; F de Rooij; C Beaumont; P Wilson; J C Deybach; Y Nordmann
Journal:  Nucleic Acids Res       Date:  1989-08-25       Impact factor: 16.971

4.  A modified spectrophotometric assay for porphobilinogen deaminase: its application in the detection of both carriers and patients with acute intermittent porphyria.

Authors:  J Vázquez-Prado; F J Sánchez-Anzaldo; G J Ruiz-Argüelles; E Marín-López; E Lobato-Mendizábal
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

5.  Prenatal exclusion of congenital erythropoietic porphyria (Günther's disease) in a fetus at risk.

Authors:  J C Deybach; B Grandchamp; M Grelier; Y Nordmann; J Boué; A Boué; P de Berrianger
Journal:  Hum Genet       Date:  1980-02       Impact factor: 4.132

6.  Direct assay of enzymes in heme biosynthesis for the detection of porphyrias by tandem mass spectrometry. Porphobilinogen deaminase.

Authors:  Yuesong Wang; C Ronald Scott; Michael H Gelb; Frantisek Turecek
Journal:  Anal Chem       Date:  2008-02-23       Impact factor: 6.986

7.  Assignment of human uroporphyrinogen I synthase locus to region 11qter by gene dosage effect.

Authors:  H de Verneuil; N Phung; Y Nordmann; D Allard; F Leprince; H Jérome; A Aurias; M O Rethoré
Journal:  Hum Genet       Date:  1982       Impact factor: 4.132

8.  In vitro CFU-E and BFU-E responses to androgen in bone marrow from children with primary hypoproliferative anaemia: a possible therapeutic assay.

Authors:  M Claustres; G Margueritte; C Sultan
Journal:  Eur J Pediatr       Date:  1986-02       Impact factor: 3.183

9.  A regulatory role for porphobilinogen deaminase (PBGD) in delta-aminolaevulinic acid (delta-ALA)-induced photosensitization?

Authors:  S L Gibson; D J Cupriks; J J Havens; M L Nguyen; R Hilf
Journal:  Br J Cancer       Date:  1998       Impact factor: 7.640

10.  Delta-aminolaevulinic acid-induced photodynamic therapy inhibits protoporphyrin IX biosynthesis and reduces subsequent treatment efficacy in vitro.

Authors:  S L Gibson; J J Havens; M L Nguyen; R Hilf
Journal:  Br J Cancer       Date:  1999-06       Impact factor: 7.640
  10 in total

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