Literature DB >> 1183475

Decreased haem synthetase activity in blood cells of patients with erythropoietic protoporphyria.

A F de Goeij, K Christianse, J van Steveninck.   

Abstract

A constant finding in erythropoietic protoporphyria (E.P.P.) is a raised protoporphyrin content of the erythrocytes. It has been shown before that the biosynthesis of this protoporphyrin takes place in the erythroid cell. In circulating blood cells of patients with E.P.P. the haem synthetase activity is significantly decreased. This observation and the increased protoporphyrin concentration in the cells can be explained by assuming a decreased stability of haem synthetase in the red blood cells of E.P.P. patients.

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Year:  1975        PMID： 1183475     DOI： 10.1111/j.1365-2362.1975.tb00470.x

Source DB:  PubMed          Journal:  Eur J Clin Invest        ISSN： 0014-2972            Impact factor:   4.686


  11 in total

1.  Study of factors causing excess protoporphyrin accumulation in cultured skin fibroblasts from patients with protoporphyria.

Authors:  J R Bloomer; D A Brenner; M J Mahoney
Journal:  J Clin Invest       Date:  1977-12       Impact factor: 14.808

2.  Photochemical damage to skin fibroblasts caused by protoporphyrin and violet light.

Authors:  A A Schothorst; C A De Haas; D Suurmond
Journal:  Arch Dermatol Res       Date:  1980       Impact factor: 3.017

3.  Cloning of murine ferrochelatase.

Authors:  D A Brenner; F Frasier
Journal:  Proc Natl Acad Sci U S A       Date:  1991-02-01       Impact factor: 11.205

4.  A molecular defect in human protoporphyria.

Authors:  D A Brenner; J M Didier; F Frasier; S R Christensen; G A Evans; H A Dailey
Journal:  Am J Hum Genet       Date:  1992-06       Impact factor: 11.025

5.  Characterization of deficient heme synthase activity in protoporphyria with cultured skin fibroblasts.

Authors:  J R Bloomer
Journal:  J Clin Invest       Date:  1980-02       Impact factor: 14.808

6.  Heme content of normal and porphyric cultured skin fibroblasts.

Authors:  D A Brenner; J R Bloomer
Journal:  Biochem Genet       Date:  1977-12       Impact factor: 1.890

7.  Bovine protoporphyria: documentation of autosomal recessive inheritance and comparison with the human disease through measurement of heme synthase activity.

Authors:  J R Bloomer; K O Morton; R J Reuter; G R Ruth
Journal:  Am J Hum Genet       Date:  1982-03       Impact factor: 11.025

8.  Uptake of protoporphyrin IX by isolated rat liver mitochondria.

Authors:  M E Koller; I Romslo
Journal:  Biochem J       Date:  1980-05-15       Impact factor: 3.857

9.  Studies in porphyria: functional evidence for a partial deficiency of ferrochelatase activity in mitogen-stimulated lymphocytes from patients with erythropoietic protoporphyria.

Authors:  S Sassa; G L Zalar; M B Poh-Fitzpatrick; K E Anderson; A Kappas
Journal:  J Clin Invest       Date:  1982-04       Impact factor: 14.808

Review 10.  Comparison of human and bovine protoporphyria.

Authors:  D A Brenner; J R Bloomer
Journal:  Yale J Biol Med       Date:  1979 Sep-Oct
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