Literature DB >> 71507

Enzyme abnormalities in the porphyrias.

M J Brodie, M R Moore, A Goldberg.   

Abstract

Evidence is presented that each of the porphyrias represents a different inborn error of metabolism in haem biosynthesis. Control of the pathway takes place by feedback repression and inhibition by haem of delta-aminolaevulinic-acid synthase. It is suggested that insituations where the activity of this enzyme is derepressed, prophobilinogen deaminase represents a secondary control step.

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Year:  1977        PMID： 71507     DOI： 10.1016/s0140-6736(77)90507-4

Source DB:  PubMed          Journal:  Lancet        ISSN： 0140-6736            Impact factor:   79.321


  12 in total

1.  Chester porphyria: a clinical study of a new form of acute porphyria.

Authors:  M R Qadiri; S E Church; K E McColl; M R Moore; G R Youngs
Journal:  Br Med J (Clin Res Ed)       Date:  1986-02-15

2.  Antipyrine metabolism in acute hepatic porphyria in relapse and remission.

Authors:  G G Birnie; K E McColl; G G Thompson; M R Moore; A Goldberg; M J Brodie
Journal:  Br J Clin Pharmacol       Date:  1987-03       Impact factor: 4.335

3.  Screening for latent acute intermittent porphyria: the value of measuring both leucocyte delta-aminolaevulinic acid synthase and erythrocyte uroporphyrinogen-1-synthase activities.

Authors:  K E McColl; M R Moore; G G Thompson; A Goldberg
Journal:  J Med Genet       Date:  1982-08       Impact factor: 6.318

4.  Effect of stanozolol on delta-aminolaevulinic acid synthase and hepatic monooxygenase activity in man and rat.

Authors:  G G Thompson; M Small; G D Lowe; C D Forbes; B K Park; G Scobie; M J Brodie
Journal:  Eur J Clin Pharmacol       Date:  1984       Impact factor: 2.953

5.  Rat hepatic uroporphyrinogen III co-synthase. Purification and evidence for a bound folate coenzyme participating in the biosynthesis of uroporphyrinogen III.

Authors:  M Kohashi; R P Clement; J Tse; W N Piper
Journal:  Biochem J       Date:  1984-06-15       Impact factor: 3.857

6.  Characterization of the porphobilinogen deaminase deficiency in acute intermittent porphyria. Immunologic evidence for heterogeneity of the genetic defect.

Authors:  P M Anderson; R M Reddy; K E Anderson; R J Desnick
Journal:  J Clin Invest       Date:  1981-07       Impact factor: 14.808

7.  The effect of fluroxene [(2,2,2-trifluoroethoxy)ethane] on haem biosynthesis and degradation.

Authors:  M R Ziman; J J Bradshaw; K M Ivanetich
Journal:  Biochem J       Date:  1980-09-15       Impact factor: 3.857

8.  The effects of chronic carbamazepine treatment of haem biosynthesis in man and rat.

Authors:  G M McGuire; G J Macphee; G G Thompson; B K Park; M R Moore; M J Brodie
Journal:  Eur J Clin Pharmacol       Date:  1988       Impact factor: 2.953

9.  Effects of cimetidine on carbamazepine auto- and hetero-induction in man.

Authors:  G J Macphee; G G Thompson; G Scobie; E Agnew; B K Park; T Murray; K E McColl; M J Brodie
Journal:  Br J Clin Pharmacol       Date:  1984-09       Impact factor: 4.335

10.  The inherited enzymatic defect in porphyria variegata.

Authors:  J C Deybach; H de Verneuil; Y Nordmann
Journal:  Hum Genet       Date:  1981       Impact factor: 4.132
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