New Insights into the Hepcidin-Ferroportin Axis and Iron Homeostasis in iPSC-Derived Cardiomyocytes from Friedreich’s Ataxia Patient
Joint Authors
Abruzzo, Provvidenza Maria
Marini, Marina
Bolotta, Alessandra
Baldassarro, Vito Antonio
Ghezzo, Alessandro
Zucchini, Cinzia
Scotlandi, Katia
Source
Oxidative Medicine and Cellular Longevity
Issue
Vol. 2019, Issue 2019 (31 Dec. 2019), pp.1-11, 11 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2019-03-27
Country of Publication
Egypt
No. of Pages
11
Main Subjects
Abstract EN
Iron homeostasis in the cardiac tissue as well as the involvement of the hepcidin-ferroportin (HAMP-FPN) axis in this process and in cardiac functionality are not fully understood.
Imbalance of iron homeostasis occurs in several cardiac diseases, including iron-overload cardiomyopathies such as Friedreich’s ataxia (FRDA, OMIM no.
229300), a hereditary neurodegenerative disorder.
Exploiting the induced pluripotent stem cells (iPSCs) technology and the iPSC capacity to differentiate into specific cell types, we derived cardiomyocytes of a FRDA patient and of a healthy control subject in order to study the cardiac iron homeostasis and the HAMP-FPN axis.
Both CTR and FRDA iPSCs-derived cardiomyocytes express cardiac differentiation markers; in addition, FRDA cardiomyocytes maintain the FRDA-like phenotype.
We found that FRDA cardiomyocytes show an increase in the protein expression of HAMP and FPN.
Moreover, immunofluorescence analysis revealed for the first time an unexpected nuclear localization of FPN in both CTR and FRDA cardiomyocytes.
However, the amount of the nuclear FPN was less in FRDA cardiomyocytes than in controls.
These and other data suggest that iron handling and the HAMP-FPN axis regulation in FRDA cardiac cells are hampered and that FPN may have new, still not fully understood, functions.
These findings underline the complexity of the cardiac iron homeostasis.
American Psychological Association (APA)
Bolotta, Alessandra& Abruzzo, Provvidenza Maria& Baldassarro, Vito Antonio& Ghezzo, Alessandro& Scotlandi, Katia& Marini, Marina…[et al.]. 2019. New Insights into the Hepcidin-Ferroportin Axis and Iron Homeostasis in iPSC-Derived Cardiomyocytes from Friedreich’s Ataxia Patient. Oxidative Medicine and Cellular Longevity،Vol. 2019, no. 2019, pp.1-11.
https://search.emarefa.net/detail/BIM-1205231
Modern Language Association (MLA)
Bolotta, Alessandra…[et al.]. New Insights into the Hepcidin-Ferroportin Axis and Iron Homeostasis in iPSC-Derived Cardiomyocytes from Friedreich’s Ataxia Patient. Oxidative Medicine and Cellular Longevity No. 2019 (2019), pp.1-11.
https://search.emarefa.net/detail/BIM-1205231
American Medical Association (AMA)
Bolotta, Alessandra& Abruzzo, Provvidenza Maria& Baldassarro, Vito Antonio& Ghezzo, Alessandro& Scotlandi, Katia& Marini, Marina…[et al.]. New Insights into the Hepcidin-Ferroportin Axis and Iron Homeostasis in iPSC-Derived Cardiomyocytes from Friedreich’s Ataxia Patient. Oxidative Medicine and Cellular Longevity. 2019. Vol. 2019, no. 2019, pp.1-11.
https://search.emarefa.net/detail/BIM-1205231
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1205231