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

American Journal of Blood Research

Patrick Lutz¹  Philippe Kastner¹  Arnaud Dupuis¹  Susan Chan¹  Marie-Pierre Gaub¹  Raoul Herbrecht¹ 

关键词: B cell leukemia;    Ikaros;    tumor suppressor;   

DOI  :  

学科分类：血液学

来源: e-Century Publishing Corporation

【 摘 要 】

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.

【 授权许可】

Unknown