Literature DB >> 905823

Bovine protoporphyria: the first nonhuman model of this hereditary photosensitizing disease.

G R RUTH, S Schwartz, B Stephenson.   

Abstract

Protoporphyria, a photosensitizing disease documented only in humans, was transmitted as a recessive trait to seven female calves. Cutaneous lesions were extensive, and erythrocyte and fecal protoporphyrin concentrations exceeded by far those of human protoporphyria. Average ferrochelatase activity was decreased to one-half of normal in the liver of carriers, and to about one-tenth of normal in liver, kidney, heart, spleen, lung, and marrow of protoporphyrics.

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Year:  1977        PMID： 905823     DOI： 10.1126/science.905823

Source DB:  PubMed          Journal:  Science        ISSN： 0036-8075            Impact factor:   47.728


  14 in total

Review 1.  Murine models of the human porphyrias: Contributions toward understanding disease pathogenesis and the development of new therapies.

Authors:  Makiko Yasuda; Robert J Desnick
Journal:  Mol Genet Metab       Date:  2019-01-18       Impact factor: 4.797

2.  Contribution of a common single-nucleotide polymorphism to the genetic predisposition for erythropoietic protoporphyria.

Authors:  Laurent Gouya; Caroline Martin-Schmitt; Anne-Marie Robreau; Frederic Austerlitz; Vasco Da Silva; Patrick Brun; Sylvie Simonin; Said Lyoumi; Bernard Grandchamp; Carole Beaumont; Herve Puy; Jean-Charles Deybach
Journal:  Am J Hum Genet       Date:  2005-11-15       Impact factor: 11.025

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

Review 4.  Porphyrias: animal models and prospects for cellular and gene therapy.

Authors:  H de Verneuil; C Ged; S Boulechfar; F Moreau-Gaudry
Journal:  J Bioenerg Biomembr       Date:  1995-04       Impact factor: 2.945

5.  Feline acute intermittent porphyria: a phenocopy masquerading as an erythropoietic porphyria due to dominant and recessive hydroxymethylbilane synthase mutations.

Authors:  Sonia Clavero; David F Bishop; Mark E Haskins; Urs Giger; Raili Kauppinen; Robert J Desnick
Journal:  Hum Mol Genet       Date:  2009-11-24       Impact factor: 6.150

6.  Erythropoietic protoporphyria in the house mouse. A recessive inherited ferrochelatase deficiency with anemia, photosensitivity, and liver disease.

Authors:  S Tutois; X Montagutelli; V Da Silva; H Jouault; P Rouyer-Fessard; K Leroy-Viard; J L Guénet; Y Nordmann; Y Beuzard; J C Deybach
Journal:  J Clin Invest       Date:  1991-11       Impact factor: 14.808

7.  Immunochemical studies of ferrochelatase protein: characterization of the normal and mutant protein in bovine and human protoporphyria.

Authors:  J G Straka; H D Hill; J M Krikava; A M Kools; J R Bloomer
Journal:  Am J Hum Genet       Date:  1991-01       Impact factor: 11.025

8.  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

9.  Protoporphyrin-induced cholestasis in the isolated in situ perfused rat liver.

Authors:  D L Avner; R G Lee; M M Berenson
Journal:  J Clin Invest       Date:  1981-02       Impact factor: 14.808

10.  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
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