Function of Ikaros as a tumor suppressor in B cell acute lymphoblastic leukemia.

Fiche publication


Date publication

janvier 2013

Auteurs

Membres identifiés du Cancéropôle Est :
Dr CHAN Susan, Pr HERBRECHT Raoul, Dr KASTNER Philippe


Tous les auteurs :
Kastner P, Dupuis A, Gaub MP, Herbrecht R, Lutz P, Chan S

Résumé

The Ikaros transcription factor is crucial for many aspects of hematopoiesis. Loss of function mutations in IKZF1, the gene encoding Ikaros, have been implicated in adult and pediatric B cell acute lymphoblastic leukemia (B-ALL). These mutations result in haploinsufficiency of the Ikaros gene in approximately half of the cases. The remaining cases contain more severe or compound mutations that lead to the generation of dominant-negative proteins or complete loss of function. All IKZF1 mutations are associated with a poor prognosis. Here we review the current genetic, clinical and mechanistic evidence for the role of Ikaros as a tumor suppressor in B-ALL.

Référence

Am J Blood Res. 2013;3(1):1-13