RARG Gene Dysregulation in Acute Myeloid Leukemia

Maria Rosa Conserva; Immacolata Redavid; Luisa Anelli; Antonella Zagaria; Giorgina Specchia; Francesco Albano

doi:10.3389/fmolb.2019.00114

RARG Gene Dysregulation in Acute Myeloid Leukemia

Front Mol Biosci. 2019 Oct 24:6:114. doi: 10.3389/fmolb.2019.00114. eCollection 2019.

Authors

Maria Rosa Conserva¹, Immacolata Redavid¹, Luisa Anelli¹, Antonella Zagaria¹, Giorgina Specchia¹, Francesco Albano¹

Affiliation

¹ Hematology Section, Department of Emergency and Organ Transplantation (D.E.T.O.), University of Bari, Bari, Italy.

Abstract

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: acute myeloid leukemia; acute promyelocytic leukemia; gene fusions; protein fusions; retinoic acid receptor γ.

Publication types

Review