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PML-RARA Fusion Protein Expression

MedGen UID:
309533
•Concept ID:
C1518798
•
Cell or Molecular Dysfunction
Synonyms: PML-RARa Fusion Protein Expression; PML-RARalpha Fusion Protein Expression; PML/RARA Fusion Protein Expression

Definition

Expression of a fusion protein that results from a fusion of the human genes PML and RARA. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPML-RARA Fusion Protein Expression

Professional guidelines

PubMed

Chemogenomics analysis of drug targets for the treatment of acute promyelocytic leukemia.
Chen S, Li X, Ma S, Xing X, Wang X, Zhu Z
Ann Hematol 2020 Apr;99(4):753-763. Epub 2020 Feb 4 doi: 10.1007/s00277-019-03888-4. PMID: 32016577
Report of a new six-panel flow cytometry marker for early differential diagnosis of APL from HLA-DR negative Non-APL leukemia.
Mosleh M, Mehrpouri M, Ghaffari S, Saei Z, Agaeipoor M, Jadali F, Satlsar ES, Gholampour R
Scand J Clin Lab Invest 2020 Feb-Apr;80(2):87-92. Epub 2019 Dec 12 doi: 10.1080/00365513.2019.1700427. PMID: 31829759
Missense mutations in PML-RARA are critical for the lack of responsiveness to arsenic trioxide treatment.
Goto E, Tomita A, Hayakawa F, Atsumi A, Kiyoi H, Naoe T
Blood 2011 Aug 11;118(6):1600-9. Epub 2011 May 25 doi: 10.1182/blood-2011-01-329433. PMID: 21613260

Recent clinical studies

Etiology

Proteogenomic analysis reveals cytoplasmic sequestration of RUNX1 by the acute myeloid leukemia-initiating CBFB::MYH11 oncofusion protein.
Day RB, Hickman JA, Xu Z, Katerndahl CD, Ferraro F, Ramakrishnan SM, Erdmann-Gilmore P, Sprung RW, Mi Y, Townsend RR, Miller CA, Ley TJ
J Clin Invest 2023 Dec 7;134(4) doi: 10.1172/JCI176311. PMID: 38061017Free PMC Article
How retinoic acid and arsenic transformed acute promyelocytic leukemia therapy.
Korsos V, Miller WH Jr
J Mol Endocrinol 2022 Nov 1;69(4):T69-T83. Epub 2022 Oct 11 doi: 10.1530/JME-22-0141. PMID: 36112505
Prognostic factors in acute promyelocytic leukemia: strategies to define high-risk patients.
Testa U, Lo-Coco F
Ann Hematol 2016 Apr;95(5):673-80. Epub 2016 Feb 26 doi: 10.1007/s00277-016-2622-1. PMID: 26920716
Dynamics of epigenetic modifications in leukemia.
Uribesalgo I, Di Croce L
Brief Funct Genomics 2011 Jan;10(1):18-29. Epub 2011 Jan 21 doi: 10.1093/bfgp/elr002. PMID: 21258047Free PMC Article
Acute leukemia in early childhood.
Emerenciano M, Koifman S, Pombo-de-Oliveira MS
Braz J Med Biol Res 2007 Jun;40(6):749-60. doi: 10.1590/s0100-879x2007000600002. PMID: 17581672

Diagnosis

How retinoic acid and arsenic transformed acute promyelocytic leukemia therapy.
Korsos V, Miller WH Jr
J Mol Endocrinol 2022 Nov 1;69(4):T69-T83. Epub 2022 Oct 11 doi: 10.1530/JME-22-0141. PMID: 36112505
Targeting PARP proteins in acute leukemia: DNA damage response inhibition and therapeutic strategies.
Padella A, Ghelli Luserna Di Rorà A, Marconi G, Ghetti M, Martinelli G, Simonetti G
J Hematol Oncol 2022 Jan 22;15(1):10. doi: 10.1186/s13045-022-01228-0. PMID: 35065680Free PMC Article
Prognostic factors in acute promyelocytic leukemia: strategies to define high-risk patients.
Testa U, Lo-Coco F
Ann Hematol 2016 Apr;95(5):673-80. Epub 2016 Feb 26 doi: 10.1007/s00277-016-2622-1. PMID: 26920716
Synergy against PML-RARa: targeting transcription, proteolysis, differentiation, and self-renewal in acute promyelocytic leukemia.
Dos Santos GA, Kats L, Pandolfi PP
J Exp Med 2013 Dec 16;210(13):2793-802. doi: 10.1084/jem.20131121. PMID: 24344243Free PMC Article
Acute leukemia in early childhood.
Emerenciano M, Koifman S, Pombo-de-Oliveira MS
Braz J Med Biol Res 2007 Jun;40(6):749-60. doi: 10.1590/s0100-879x2007000600002. PMID: 17581672

Therapy

Function of PML-RARA in Acute Promyelocytic Leukemia.
Testa U, Pelosi E
Adv Exp Med Biol 2024;1459:321-339. doi: 10.1007/978-3-031-62731-6_14. PMID: 39017850
How retinoic acid and arsenic transformed acute promyelocytic leukemia therapy.
Korsos V, Miller WH Jr
J Mol Endocrinol 2022 Nov 1;69(4):T69-T83. Epub 2022 Oct 11 doi: 10.1530/JME-22-0141. PMID: 36112505
MicroRNA-125b Accelerates and Promotes PML-RARa-driven Murine Acute Promyelocytic Leukemia.
Guo B, Qin R, Chen JJ, Pan W, Lu XC
Biomed Environ Sci 2022 Jun 20;35(6):485-493. doi: 10.3967/bes2022.067. PMID: 35882408
Targeting PARP proteins in acute leukemia: DNA damage response inhibition and therapeutic strategies.
Padella A, Ghelli Luserna Di Rorà A, Marconi G, Ghetti M, Martinelli G, Simonetti G
J Hematol Oncol 2022 Jan 22;15(1):10. doi: 10.1186/s13045-022-01228-0. PMID: 35065680Free PMC Article
RARA and PML genes in acute promyelocytic leukemia.
Chen Z, Chen SJ
Leuk Lymphoma 1992 Nov;8(4-5):253-60. doi: 10.3109/10428199209051004. PMID: 1337847

Prognosis

Genetic fusions favor tumorigenesis through degron loss in oncogenes.
Liu J, Tokheim C, Lee JD, Gan W, North BJ, Liu XS, Pandolfi PP, Wei W
Nat Commun 2021 Nov 18;12(1):6704. doi: 10.1038/s41467-021-26871-y. PMID: 34795215Free PMC Article
Chemogenomics analysis of drug targets for the treatment of acute promyelocytic leukemia.
Chen S, Li X, Ma S, Xing X, Wang X, Zhu Z
Ann Hematol 2020 Apr;99(4):753-763. Epub 2020 Feb 4 doi: 10.1007/s00277-019-03888-4. PMID: 32016577
Prognostic factors in acute promyelocytic leukemia: strategies to define high-risk patients.
Testa U, Lo-Coco F
Ann Hematol 2016 Apr;95(5):673-80. Epub 2016 Feb 26 doi: 10.1007/s00277-016-2622-1. PMID: 26920716
Varying responses of PML-RARA with different genetic mutations to arsenic trioxide.
Liu J, Zhu HH, Jiang H, Jiang Q, Huang XJ
Blood 2016 Jan 14;127(2):243-50. Epub 2015 Nov 4 doi: 10.1182/blood-2015-04-637678. PMID: 26537301
Acute promyelocytic leukemia: a paradigm for differentiation therapy.
Grimwade D, Mistry AR, Solomon E, Guidez F
Cancer Treat Res 2010;145:219-35. doi: 10.1007/978-0-387-69259-3_13. PMID: 20306254

Clinical prediction guides

MicroRNA-125b Accelerates and Promotes PML-RARa-driven Murine Acute Promyelocytic Leukemia.
Guo B, Qin R, Chen JJ, Pan W, Lu XC
Biomed Environ Sci 2022 Jun 20;35(6):485-493. doi: 10.3967/bes2022.067. PMID: 35882408
Targeting PARP proteins in acute leukemia: DNA damage response inhibition and therapeutic strategies.
Padella A, Ghelli Luserna Di Rorà A, Marconi G, Ghetti M, Martinelli G, Simonetti G
J Hematol Oncol 2022 Jan 22;15(1):10. doi: 10.1186/s13045-022-01228-0. PMID: 35065680Free PMC Article
Genetic fusions favor tumorigenesis through degron loss in oncogenes.
Liu J, Tokheim C, Lee JD, Gan W, North BJ, Liu XS, Pandolfi PP, Wei W
Nat Commun 2021 Nov 18;12(1):6704. doi: 10.1038/s41467-021-26871-y. PMID: 34795215Free PMC Article
Chemogenomics analysis of drug targets for the treatment of acute promyelocytic leukemia.
Chen S, Li X, Ma S, Xing X, Wang X, Zhu Z
Ann Hematol 2020 Apr;99(4):753-763. Epub 2020 Feb 4 doi: 10.1007/s00277-019-03888-4. PMID: 32016577
Prognostic factors in acute promyelocytic leukemia: strategies to define high-risk patients.
Testa U, Lo-Coco F
Ann Hematol 2016 Apr;95(5):673-80. Epub 2016 Feb 26 doi: 10.1007/s00277-016-2622-1. PMID: 26920716

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