Altered Erythropoiesis in Mouse Models of Type 3 Hemochromatosis
Joint Authors
Pellegrino, R. M.
Ferbo, L.
Boero, M.
Palmieri, A.
Osella, L.
Pollicino, P.
Miniscalco, B.
Roetto, A.
Riondato, Fulvio
Saglio, Giuseppe G.
Source
Issue
Vol. 2017, Issue 2017 (31 Dec. 2017), pp.1-12, 12 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2017-05-02
Country of Publication
Egypt
No. of Pages
12
Main Subjects
Abstract EN
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 (Tfr2α and Tfr2β).
Tfr2α is one of the hepatic regulators of iron inhibitor hepcidin.
Tfr2β is 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 Tfr2α leads 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 Tfr2α in modulation of erythropoiesis and of Tfr2β in favoring iron availability for erythropoiesis.
American Psychological Association (APA)
Pellegrino, R. M.& Riondato, Fulvio& Ferbo, L.& Boero, M.& Palmieri, A.& Osella, L.…[et al.]. 2017. Altered Erythropoiesis in Mouse Models of Type 3 Hemochromatosis. BioMed Research International،Vol. 2017, no. 2017, pp.1-12.
https://search.emarefa.net/detail/BIM-1134713
Modern Language Association (MLA)
Pellegrino, R. M.…[et al.]. Altered Erythropoiesis in Mouse Models of Type 3 Hemochromatosis. BioMed Research International No. 2017 (2017), pp.1-12.
https://search.emarefa.net/detail/BIM-1134713
American Medical Association (AMA)
Pellegrino, R. M.& Riondato, Fulvio& Ferbo, L.& Boero, M.& Palmieri, A.& Osella, L.…[et al.]. Altered Erythropoiesis in Mouse Models of Type 3 Hemochromatosis. BioMed Research International. 2017. Vol. 2017, no. 2017, pp.1-12.
https://search.emarefa.net/detail/BIM-1134713
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1134713