Publikationsdatenbank

The pathology of comparative animal models of human haemochromatosis (2012)

Art

Zeitschriftenartikel / wissenschaftlicher Beitrag

Autoren

Klopfleisch, R

Olias, P

Quelle

Journal of comparative pathology

Bandzählung: 147

Heftzählung: 4

Seiten: 460 – 478

ISSN: 0021-9975

Sprache

Englisch

Verweise

DOI: 10.1016/j.jcpa.2012.09.001

Pubmed: 23079103

Kontakt

Institut für Tierpathologie

Robert-von-Ostertag-Str. 15
14163 Berlin
+49 30 838 62450
pathologie@vetmed.fu-berlin.de

Abstract / Zusammenfassung

Haemochromatosis is one of the most common human hereditary diseases. It is defined as a pathological condition with normal iron-driven erythropoiesis, but toxic accumulation of iron in vital organs, which is caused by mutations in any gene that encodes a protein involved in limiting the entry of iron into the blood. Iron storage diseases have also been described in several mammalian and avian species and these have been proposed as comparative animal models for human haemochromatosis. Genetically engineered mouse strains with mutations in iron metabolism genes model several aspects of human haemochromatosis and study of these animals has facilitated understanding of the disease. Spontaneously arising iron storage diseases in non-murine species also overlap in some clinicopathological aspects with human haemochromatosis. However, the lack of conclusive information on the molecular biology of theses species-specific diseases and the common impact of dietary iron concentration on disease progression in most species limit their usefulness as comparative models.