The role of NPM1 alternative splicing in patients with chronic lymphocytic leukemia.


Journal

PloS one
ISSN: 1932-6203
Titre abrégé: PLoS One
Pays: United States
ID NLM: 101285081

Informations de publication

Date de publication:
2022
Historique:
received: 30 05 2022
accepted: 11 10 2022
entrez: 25 10 2022
pubmed: 26 10 2022
medline: 28 10 2022
Statut: epublish

Résumé

Chronic lymphocytic leukemia (CLL) is a lymphoproliferative disease with heterogeneous clinical course. Recent studies revealed a link between NOTCH1 mutation and the overexpression of MYC and MYC-related genes involved in ribosome biogenesis and protein biosynthesis, such as nucleophosmin-1 (NPM1), in CLL cells. In the present study, we aim to evaluate the impact of the NOTCH1 mutation on the MYC and MYC induced NPM1 expression in CLL cells via quantification of their transcripts. Using qRT-PCR, we analyzed the levels of MYC and three main NPM1 splice variants in 214 samples collected from CLL patients. We assessed the impact of each splice variant on CLL prognostic markers, including the IGHV, TP53, NOTCH1, SF3B1, and MYD88 mutational status, cytogenetic aberrations, and laboratory features. Significantly higher levels of NPM1.R1 transcripts in patients with unmutated compared to mutated IGHV status were found. The median time to first treatment (TTFT) in patients with a high level of NPM1.R1 was significantly shorter compared to the group with low NPM1.R1 levels (1.5 vs 33 months, p = 0.0002). Moreover, in Multivariate Cox Proportional Hazard Regression Model NPM1.R1 splice variant provided an independent prognostic value for TTFT. In conclusion, our study indicates the prognostic significance of the level of NPM1.R1 expression and suggests the importance of splicing alterations in the pathogenesis of CLL.

Identifiants

pubmed: 36282861
doi: 10.1371/journal.pone.0276674
pii: PONE-D-22-15632
pmc: PMC9595542
doi:

Substances chimiques

Myeloid Differentiation Factor 88 0
Nuclear Proteins 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

e0276674

Déclaration de conflit d'intérêts

The authors have declared that no competing interests exist.

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Auteurs

Monika Szelest (M)

Department of Experimental Hematooncology, Medical University of Lublin, Lublin, Poland.

Marta Masternak (M)

Department of Experimental Hematooncology, Medical University of Lublin, Lublin, Poland.
Department of Hematology, St. John's Cancer Centre, Lublin, Poland.

Małgorzata Zając (M)

Department of Experimental Hematooncology, Medical University of Lublin, Lublin, Poland.

Michał Chojnacki (M)

Department of Experimental Hematooncology, Medical University of Lublin, Lublin, Poland.

Katarzyna Skórka (K)

Department of Experimental Hematooncology, Medical University of Lublin, Lublin, Poland.

Joanna Zaleska (J)

Department of Experimental Hematooncology, Medical University of Lublin, Lublin, Poland.

Agnieszka Karczmarczyk (A)

Department of Experimental Hematooncology, Medical University of Lublin, Lublin, Poland.

Grażyna Stasiak (G)

Department of Experimental Hematooncology, Medical University of Lublin, Lublin, Poland.

Ewa Wawrzyniak (E)

Department of Hematology, Medical University of Lodz, Lodz, Poland.

Aleksandra Kotkowska (A)

Department of Hematology, Medical University of Lodz, Lodz, Poland.

Monika Siemieniuk-Ryś (M)

Department of Hematology, Medical University of Lodz, Lodz, Poland.

Joanna Purkot (J)

Department of Experimental Hematooncology, Medical University of Lublin, Lublin, Poland.

Edyta Subocz (E)

Department of Hematology, Military Institute of Medicine, Warsaw, Poland.
Independent Public Health Care Center of the Ministry of Internal Affairs and Administration with the Warmian-Masurian Oncology Centre in Olsztyn, Olsztyn, Poland.

Edyta Cichocka (E)

Department of Hematology, Copernicus Hospital, Torun, Poland.

Waldemar Tomczak (W)

Department of Hematooncology and Bone Marrow Transplantation Unit, Medical University of Lublin, Lublin, Poland.

Daria Zawirska (D)

Department of Hematology, Jagiellonian University, Krakow, Poland.

Krzysztof Giannopoulos (K)

Department of Experimental Hematooncology, Medical University of Lublin, Lublin, Poland.
Department of Hematology, St. John's Cancer Centre, Lublin, Poland.

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Classifications MeSH