Function of Ikaros as a tumor suppressor in B cell acute lymphoblastic leukemia.
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.
Institut de Génétique et de Biologie Moléculaire et Cellulaire (IGBMC), INSERM U964, CNRS UMR 7104, Université de Strasbourg 67404 Illkirch, France ; Université de Strasbourg, Faculté de Médecine Strasbourg, France.
Philippe
P
Kastner
Arnaud
A
Dupuis
Marie-Pierre
MP
Gaub
Raoul
R
Herbrecht
Patrick
P
Lutz
Susan
S
Chan
B cell leukemia; Ikaros; tumor suppressor