Alteration of NOTCH1 in T-cell Acute Lymphoblastic Leukemia and Development of Target Therapeutic Agent / 임상소아혈액종양
Clinical Pediatric Hematology-Oncology
; : 1-8, 2014.
Article
en Ko
| WPRIM
| ID: wpr-53111
Biblioteca responsable:
WPRO
ABSTRACT
T-cell acute lymphoblastic leukemia (T-ALL) accounts for approximately 10-15% of entire ALL in children. The outcome of T-ALL has been improved through the intensified therapeutic strategy, however, it is still a more aggressive disease. In T-ALL a couple of transcription factor oncogenes are known to be relocated to the juxtaposition of T-cell receptor genes, potent promoter, by chromosome translocation. However the incidence of each chimeric gene formation in T-ALL is less than 5% and their clinical significance as a prognostic marker is lacking. A decade ago it was identified that activating mutations in NOTCH1 in about 60% of T-ALL. After then, activating NOTCH1 mutations present in T-ALL have been extensively investigated with regard to understanding its molecular pathogenesis, its prognostic significance, and developing molecularly tailored novel agents. Small molecule gamma-secretase inhibitor, blocking a proteolytic step required for creation of a fragment of NOTCH intracellular domain which actually act as a controller of its target gene expression, was tried as a target therapeutic drug for T-ALL. Although outcome of this drug was not satisfactory, challenges have been launched to develop new drugs which specifically act on the aberrant behavior of mutated NOTCH1 in T-ALL.
Palabras clave
Texto completo:
1
Índice:
WPRIM
Asunto principal:
Oncogenes
/
Factores de Transcripción
/
Linfocitos T
/
Expresión Génica
/
Incidencia
/
Genes Codificadores de los Receptores de Linfocitos T
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Secretasas de la Proteína Precursora del Amiloide
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Leucemia-Linfoma Linfoblástico de Células Precursoras
/
Leucemia-Linfoma Linfoblástico de Células T Precursoras
Tipo de estudio:
Incidence_studies
/
Prognostic_studies
Límite:
Child
/
Humans
Idioma:
Ko
Revista:
Clinical Pediatric Hematology-Oncology
Año:
2014
Tipo del documento:
Article