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The Functional Versatility of Transferrin Receptor 2 and Its Therapeutic Value
Iron homeostasis is a tightly regulated process in all living organisms because this metal is essential for cellular metabolism, but could be extremely toxic when present in excess. In mammals, there is a complex pathway devoted to iron regulation, whose key protein is hepcidin (Hepc), which is a po...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6316356/ https://www.ncbi.nlm.nih.gov/pubmed/30360575 http://dx.doi.org/10.3390/ph11040115 |
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author | Roetto, Antonella Mezzanotte, Mariarosa Pellegrino, Rosa Maria |
author_facet | Roetto, Antonella Mezzanotte, Mariarosa Pellegrino, Rosa Maria |
author_sort | Roetto, Antonella |
collection | PubMed |
description | Iron homeostasis is a tightly regulated process in all living organisms because this metal is essential for cellular metabolism, but could be extremely toxic when present in excess. In mammals, there is a complex pathway devoted to iron regulation, whose key protein is hepcidin (Hepc), which is a powerful iron absorption inhibitor mainly produced by the liver. Transferrin receptor 2 (Tfr2) is one of the hepcidin regulators, and mutations in TFR2 gene are responsible for type 3 hereditary hemochromatosis (HFE3), a genetically heterogeneous disease characterized by systemic iron overload. It has been recently pointed out that Hepc production and iron regulation could be exerted also in tissues other than liver, and that Tfr2 has an extrahepatic role in iron metabolism as well. This review summarizes all the most recent data on Tfr2 extrahepatic role, taking into account the putative distinct roles of the two main Tfr2 isoforms, Tfr2α and Tfr2β. Representing Hepc modulation an effective approach to correct iron balance impairment in common human diseases, and with Tfr2 being one of its regulators, it would be worthwhile to envisage Tfr2 as a therapeutic target. |
format | Online Article Text |
id | pubmed-6316356 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-63163562019-01-11 The Functional Versatility of Transferrin Receptor 2 and Its Therapeutic Value Roetto, Antonella Mezzanotte, Mariarosa Pellegrino, Rosa Maria Pharmaceuticals (Basel) Review Iron homeostasis is a tightly regulated process in all living organisms because this metal is essential for cellular metabolism, but could be extremely toxic when present in excess. In mammals, there is a complex pathway devoted to iron regulation, whose key protein is hepcidin (Hepc), which is a powerful iron absorption inhibitor mainly produced by the liver. Transferrin receptor 2 (Tfr2) is one of the hepcidin regulators, and mutations in TFR2 gene are responsible for type 3 hereditary hemochromatosis (HFE3), a genetically heterogeneous disease characterized by systemic iron overload. It has been recently pointed out that Hepc production and iron regulation could be exerted also in tissues other than liver, and that Tfr2 has an extrahepatic role in iron metabolism as well. This review summarizes all the most recent data on Tfr2 extrahepatic role, taking into account the putative distinct roles of the two main Tfr2 isoforms, Tfr2α and Tfr2β. Representing Hepc modulation an effective approach to correct iron balance impairment in common human diseases, and with Tfr2 being one of its regulators, it would be worthwhile to envisage Tfr2 as a therapeutic target. MDPI 2018-10-23 /pmc/articles/PMC6316356/ /pubmed/30360575 http://dx.doi.org/10.3390/ph11040115 Text en © 2018 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Roetto, Antonella Mezzanotte, Mariarosa Pellegrino, Rosa Maria The Functional Versatility of Transferrin Receptor 2 and Its Therapeutic Value |
title | The Functional Versatility of Transferrin Receptor 2 and Its Therapeutic Value |
title_full | The Functional Versatility of Transferrin Receptor 2 and Its Therapeutic Value |
title_fullStr | The Functional Versatility of Transferrin Receptor 2 and Its Therapeutic Value |
title_full_unstemmed | The Functional Versatility of Transferrin Receptor 2 and Its Therapeutic Value |
title_short | The Functional Versatility of Transferrin Receptor 2 and Its Therapeutic Value |
title_sort | functional versatility of transferrin receptor 2 and its therapeutic value |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6316356/ https://www.ncbi.nlm.nih.gov/pubmed/30360575 http://dx.doi.org/10.3390/ph11040115 |
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