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Prognostic Significance ofNOTCH1andFBXW7Mutations in Pediatric T-ALL

Authors: Omer Devecioglu; Omer Dogru; Zafer Salcioglu; Sema Anak; Gülen Tüysüz; Yucel Erbilgin; Tiraje Celkan; +10 Authors

Prognostic Significance ofNOTCH1andFBXW7Mutations in Pediatric T-ALL

Abstract

The NOTCH signaling pathway plays important role in the development of multicellular organisms, as it regulates cell proliferation, survival, and differentiation. In adults, it is essential for the T- or B-lymphocyte lineage commitment.NOTCH1and FBXW7 mutations both lead the activation of theNOTCH1pathway and are found in the majority of T-ALL patients. In this study, the mutation analysis ofNOTCH1andFBXW7genes was performed in 87 pediatric T-ALLs who were treated on the ALL-BFM protocols. In 19 patients (22%), activatingNOTCH1mutations were observed either in the heterodimerization domain or in the PEST domain and 7 cases (10%) demonstrated FBXW7 mutations (2 cases had bothNOTCH1andFBXW7mutations). We also analyzed the relationship of the mutation data between the clinical and biological data of the patients.NOTCH1andFBXW7,NOTCH1alone were found correlated with lower initial leucocyte counts which was independent from the sex and T- cell immunophenotype. However,NOTCH1andFBXW7mutations were not predictive of outcome in the overall cohort of pediatric T-ALLs.

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Keywords

Male, F-Box-WD Repeat-Containing Protein 7, Adolescent, Ubiquitin-Protein Ligases, Cell Cycle Proteins, Precursor T-Cell Lymphoblastic Leukemia-Lymphoma, Humans, RNA, Messenger, RNA, Neoplasm, Receptor, Notch1, Child, Reverse Transcriptase Polymerase Chain Reaction, F-Box Proteins, Infant, Newborn, Infant, Prognosis, Survival Rate, Child, Preschool, Mutation, Female, Other

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    Top 10%
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citations
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
29
Top 10%
Top 10%
Top 10%
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