Down Regulation of Hepcidin and Haemojuvelin Expression in the Hepatocyte Cell-Line HepG2 Induced by Thalassaemic Sera
| Autor: | Ariel Koren, Konstantin Adamsky, Stefano Rivella, Ninette Amariglio, Gideon Rechavi, Carina Levin, William Breuer, Eliezer A. Rachmilewitz, Sara Gardenghi, Yoav Zvi Cabantchik, Orly Weizer-Stern |
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| Rok vydání: | 2006 |
| Předmět: |
Ineffective erythropoiesis
congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty education.field_of_study biology Transferrin saturation Immunology Beta thalassemia Cell Biology Hematology medicine.disease medicine.disease_cause Biochemistry Endocrinology medicine.anatomical_structure Hepcidin Oncogene 24p3 hemic and lymphatic diseases Internal medicine Hepatocyte medicine biology.protein HAMP education Hemochromatosis |
| Zdroj: | Blood. 108:1556-1556 |
| ISSN: | 1528-0020 0006-4971 |
| DOI: | 10.1182/blood.v108.11.1556.1556 |
| Popis: | β-thalassaemia represents a group of diseases, in which ineffective erythropoiesis is accompanied by iron overload. In a mouse model of β-thalassaemia we observed that the liver expresses relatively low levels of hepcidin, which is a key factor in the regulation of iron absorption by the gut and of iron recycling by the reticuloendothelial system. We hypothesized that despite the overt iron overload, a putative plasma factor found in β-thalassaemia might suppress liver hepcidin expression. We therefore compared sera from β-thalassaemia and haemochromatosis (C282Y mutation) patients with those of healthy individuals in terms of their capacity to evoke changes in expression of key genes of iron metabolism in human HepG2 hepatoma cells. Sera from β-thalassaemia major patients evoked a major decrease in hepcidin (HAMP) and lipocalin2 (oncogene 24p3) (LCN2) expression, as well as a moderate decrease in haemojuvelin (HFE2) expression, compared to sera from healthy individuals. Significant correlation was found between the degree of downregulation of HAMP and HFE2 evoked by b-thalassaemia major sera (r=0.852, p |
| Databáze: | OpenAIRE |
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