Altered Erythropoiesis in Mouse Models of Type 3 Hemochromatosis
| Autor: | Ludovica Ferbo, Antonella Roetto, Rosa Maria Pellegrino, Martina Boero, Fulvio Riondato, Giuseppe Saglio, Barbara Miniscalco, Luca Osella, Paolo Pollicino, Antonietta Palmieri |
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| Jazyk: | angličtina |
| Rok vydání: | 2017 |
| Předmět: |
0301 basic medicine
medicine.medical_specialty Article Subject Iron lcsh:Medicine Transferrin receptor Spleen Macrocytosis General Biochemistry Genetics and Molecular Biology Mice 03 medical and health sciences Reticulocyte Hepcidin Internal medicine Receptors Transferrin medicine Animals Protein Isoforms Erythropoiesis Mononuclear Phagocyte System Hemochromatosis Mice Knockout General Immunology and Microbiology biology lcsh:R General Medicine medicine.disease Disease Models Animal 030104 developmental biology medicine.anatomical_structure Endocrinology Liver biology.protein Bone marrow Research Article |
| Zdroj: | BioMed Research International BioMed Research International, Vol 2017 (2017) |
| Popis: | Type 3 haemochromatosis (HFE3) is a rare genetic iron overload disease which ultimately lead to compromised organs functioning. HFE3 is caused by mutations in transferrin receptor 2 (TFR2) gene that codes for two main isoforms (Tfr2αand Tfr2β). Tfr2αis one of the hepatic regulators of iron inhibitor hepcidin. Tfr2βis an intracellular isoform of the protein involved in the regulation of iron levels in reticuloendothelial cells. It has been recently demonstrated that Tfr2 is also involved in erythropoiesis. This study aims to further investigate Tfr2 erythropoietic role by evaluating the erythropoiesis of two Tfr2 murine models wherein either one or both of Tfr2 isoforms have been selectively silenced (Tfr2 KI and Tfr2 KO). The evaluations were performed in bone marrow and spleen, in 14 days’ and 10 weeks’ old mice, to assess erythropoiesis in young versus adult animals. The lack of Tfr2αleads to macrocytosis with low reticulocyte number and increased hemoglobin values, together with an anticipation of adult BM erythropoiesis and an increased splenic erythropoiesis. On the other hand, lack of Tfr2β(Tfr2 KI mice) causes an increased and immature splenic erythropoiesis. Taken together, these data confirm the role of Tfr2αin modulation of erythropoiesis and of Tfr2βin favoring iron availability for erythropoiesis. |
| Databáze: | OpenAIRE |
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