RARG gene dysregulation in acute myeloid leukemia
- 1Department of Emergency and Organ Transplantation, School of Medicine, University of Bari Aldo Moro, Italy
Retinoic acid receptor γ (RARγ) belongs to the nuclear receptor superfamily and shares 90% homology with retinoic acid receptor α (RARα) and retinoic acid receptor β (RARβ). RARA rearrangements are well known to be involved in acute promyelocytic leukemia (APL), but RARG rearrangements can also resemble this kind of leukemia. In this review we trace the role of RARγ, considering both its physiological and oncogenic contribution; from 2011 to date, nine cases of patients harboring RARG fusions have been reported. These patients showed typical APL features, including the clinical presentation, coagulation abnormalities and morphological features of bone marrow (BM), but are not responsive to APL standard therapy. We stress the urgent need for a better comprehension of the critical role of RARG dysregulation in the leukemogenesis process, since optimum therapy strategies have not yet been established.
Keywords: Retinoic acid receptor γ, Acute promyelocytic leukemia, Acute Myeloid Leukemia, Gene fusions, Protein fusions
Received: 08 Jul 2019;
Accepted: 10 Oct 2019.
Copyright: © 2019 Conserva, Redavid, Anelli, Zagaria, Specchia and Albano. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Prof. Francesco Albano, Department of Emergency and Organ Transplantation, School of Medicine, University of Bari Aldo Moro, Bari, 70124, Italy, firstname.lastname@example.org