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RARG Gene Dysregulation in Acute Myeloid Leukemia
- Source :
- Frontiers in Molecular Biosciences, Frontiers in Molecular Biosciences, Vol 6 (2019)
- Publication Year :
- 2019
- Publisher :
- Frontiers Media S.A., 2019.
-
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.
- Subjects :
- 0301 basic medicine
Acute promyelocytic leukemia
retinoic acid receptor γ
Review
Biology
acute myeloid leukemia
Biochemistry, Genetics and Molecular Biology (miscellaneous)
Biochemistry
Homology (biology)
gene fusions
03 medical and health sciences
0302 clinical medicine
medicine
Molecular Biosciences
lcsh:QH301-705.5
Molecular Biology
Gene
Myeloid leukemia
acute promyelocytic leukemia
medicine.disease
Leukemia
Retinoic acid receptor
030104 developmental biology
medicine.anatomical_structure
lcsh:Biology (General)
Nuclear receptor
030220 oncology & carcinogenesis
Cancer research
protein fusions
Bone marrow
Subjects
Details
- Language :
- English
- ISSN :
- 2296889X
- Volume :
- 6
- Database :
- OpenAIRE
- Journal :
- Frontiers in Molecular Biosciences
- Accession number :
- edsair.doi.dedup.....8dc5d9b24f38959500d32484350f9a91