title: Notch in T-ALL: new players in a complex disease.
abstract: T cell acute lymphoblastic leukemia (T-ALL) is an aggressive hematopoietic malignancy of thymocytes affecting preferentially children and adolescents. The disease is heterogeneous and characterized by a large set of chromosomal and genetic alterations that deregulate the growth of maturing thymocytes. The identification of activating point mutations in NOTCH1 in more then 50% of all T-ALL cases highlights the NOTCH1 cascade as a central player of T-ALL pathogenesis. In this review, we summarize and update more recent findings on the molecular mechanisms of T-ALL with a particular emphasis on the oncogenic properties of aberrant NOTCH1 signaling.
authors.affiliation: Ecole Polytechnique Fédérale de Lausanne, Swiss Institute for Experimental Cancer Research (ISREC), Station 19, 1015 Lausanne, Switzerland.
authors.forename: Ute
authors.initials: U
authors.lastname: Koch
authors.forename: Freddy
authors.initials: F
authors.lastname: Radtke
mesh_terms: D000293:Adolescent; D000818:Animals; D002454:Cell Differentiation / Q000235:genetics / Q000276:immunology; D002648:Child; D015973:Gene Expression Regulation, Leukemic / Q000276:immunology*; D053263:Gene Regulatory Networks / Q000276:immunology*; D019672:Genes, T-Cell Receptor; D006801:Humans; D051379:Mice; D035683:MicroRNAs / Q000276:immunology / Q000378:metabolism; D058990:Molecular Targeted Therapy; D009363:Neoplasm Proteins / Q000235:genetics / Q000276:immunology / Q000378:metabolism; D017354:Point Mutation; D054218:Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D051881:Receptor, Notch1 / Q000235:genetics / Q000276:immunology* / Q000378:metabolism; D015398:Signal Transduction / Q000235:genetics / Q000276:immunology*; D013045:Species Specificity; D013601:T-Lymphocytes / Q000166:cytology / Q000276:immunology / Q000378:metabolism; D060168:Thymocytes / Q000276:immunology* / Q000378:metabolism / Q000473:pathology; D014178:Translocation, Genetic
chemical_list: D035683:MicroRNAs; D009363:Neoplasm Proteins; D051881:Receptor, Notch1