NOTCH1 Aberrations in Chronic Lymphocytic Leukemia
Chronic lymphocytic leukemia (CLL) is an incurable B-cell neoplasm characterized by highly variable clinical outcomes. In recent years, genomic and molecular studies revealed a remarkable heterogeneity in CLL, which mirrored the clinical diversity of this disease. These studies profoundly enhanced o...
| Main Authors: | , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2018-06-01
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| Series: | Frontiers in Oncology |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/article/10.3389/fonc.2018.00229/full |
| _version_ | 1811293446443368448 |
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| author | Emanuela Rosati Stefano Baldoni Filomena De Falco Beatrice Del Papa Erica Dorillo Chiara Rompietti Elisa Albi Franca Falzetti Mauro Di Ianni Mauro Di Ianni Paolo Sportoletti |
| author_facet | Emanuela Rosati Stefano Baldoni Filomena De Falco Beatrice Del Papa Erica Dorillo Chiara Rompietti Elisa Albi Franca Falzetti Mauro Di Ianni Mauro Di Ianni Paolo Sportoletti |
| author_sort | Emanuela Rosati |
| collection | DOAJ |
| description | Chronic lymphocytic leukemia (CLL) is an incurable B-cell neoplasm characterized by highly variable clinical outcomes. In recent years, genomic and molecular studies revealed a remarkable heterogeneity in CLL, which mirrored the clinical diversity of this disease. These studies profoundly enhanced our understanding of leukemia cell biology and led to the identification of new biomarkers with potential prognostic and therapeutic significance. Accumulating evidence indicates a key role of deregulated NOTCH1 signaling and NOTCH1 mutations in CLL. This review highlights recent discoveries that improve our understanding of the pathophysiological NOTCH1 signaling in CLL and the clinical impact of NOTCH1 mutations in retrospective and prospective trials. In addition, we discuss the rationale for a therapeutic strategy aiming at inhibiting NOTCH1 signaling in CLL, along with an overview on the currently available NOTCH1-directed approaches. |
| first_indexed | 2024-04-13T05:01:31Z |
| format | Article |
| id | doaj.art-36d9f649ddc84b009499ec08ae48b5ad |
| institution | Directory Open Access Journal |
| issn | 2234-943X |
| language | English |
| last_indexed | 2024-04-13T05:01:31Z |
| publishDate | 2018-06-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Oncology |
| spelling | doaj.art-36d9f649ddc84b009499ec08ae48b5ad2022-12-22T03:01:18ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2018-06-01810.3389/fonc.2018.00229384747NOTCH1 Aberrations in Chronic Lymphocytic LeukemiaEmanuela Rosati0Stefano Baldoni1Filomena De Falco2Beatrice Del Papa3Erica Dorillo4Chiara Rompietti5Elisa Albi6Franca Falzetti7Mauro Di Ianni8Mauro Di Ianni9Paolo Sportoletti10Department of Experimental Medicine, Biosciences and Medical Embryology Section, University of Perugia, Perugia, ItalyDepartment of Life, Hematology Section, Health and Environmental Sciences, University of L’Aquila, Perugia, ItalyInstitute of Hematology-Centro di Ricerche Emato-Oncologiche (CREO), University of Perugia, Perugia, ItalyInstitute of Hematology-Centro di Ricerche Emato-Oncologiche (CREO), University of Perugia, Perugia, ItalyInstitute of Hematology-Centro di Ricerche Emato-Oncologiche (CREO), University of Perugia, Perugia, ItalyInstitute of Hematology-Centro di Ricerche Emato-Oncologiche (CREO), University of Perugia, Perugia, ItalyInstitute of Hematology-Centro di Ricerche Emato-Oncologiche (CREO), University of Perugia, Perugia, ItalyInstitute of Hematology-Centro di Ricerche Emato-Oncologiche (CREO), University of Perugia, Perugia, ItalyDepartment of Medicine and Aging Sciences, University of Chieti Pescara, Chieti, ItalyDepartment of Hematology, Transfusion Medicine and Biotechnologies, Ospedale Civile, Pescara, ItalyInstitute of Hematology-Centro di Ricerche Emato-Oncologiche (CREO), University of Perugia, Perugia, ItalyChronic lymphocytic leukemia (CLL) is an incurable B-cell neoplasm characterized by highly variable clinical outcomes. In recent years, genomic and molecular studies revealed a remarkable heterogeneity in CLL, which mirrored the clinical diversity of this disease. These studies profoundly enhanced our understanding of leukemia cell biology and led to the identification of new biomarkers with potential prognostic and therapeutic significance. Accumulating evidence indicates a key role of deregulated NOTCH1 signaling and NOTCH1 mutations in CLL. This review highlights recent discoveries that improve our understanding of the pathophysiological NOTCH1 signaling in CLL and the clinical impact of NOTCH1 mutations in retrospective and prospective trials. In addition, we discuss the rationale for a therapeutic strategy aiming at inhibiting NOTCH1 signaling in CLL, along with an overview on the currently available NOTCH1-directed approaches.https://www.frontiersin.org/article/10.3389/fonc.2018.00229/fullNOTCH1chronic lymphocytic leukemiaprognostic biomarkertargeted therapygene mutation |
| spellingShingle | Emanuela Rosati Stefano Baldoni Filomena De Falco Beatrice Del Papa Erica Dorillo Chiara Rompietti Elisa Albi Franca Falzetti Mauro Di Ianni Mauro Di Ianni Paolo Sportoletti NOTCH1 Aberrations in Chronic Lymphocytic Leukemia Frontiers in Oncology NOTCH1 chronic lymphocytic leukemia prognostic biomarker targeted therapy gene mutation |
| title | NOTCH1 Aberrations in Chronic Lymphocytic Leukemia |
| title_full | NOTCH1 Aberrations in Chronic Lymphocytic Leukemia |
| title_fullStr | NOTCH1 Aberrations in Chronic Lymphocytic Leukemia |
| title_full_unstemmed | NOTCH1 Aberrations in Chronic Lymphocytic Leukemia |
| title_short | NOTCH1 Aberrations in Chronic Lymphocytic Leukemia |
| title_sort | notch1 aberrations in chronic lymphocytic leukemia |
| topic | NOTCH1 chronic lymphocytic leukemia prognostic biomarker targeted therapy gene mutation |
| url | https://www.frontiersin.org/article/10.3389/fonc.2018.00229/full |
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