Am J Blood Res 2013;3(1):1-13
Review Article
Function of Ikaros as a tumor suppressor in B cell acute lympho-blastic
leukemia
Philippe Kastner, Arnaud Dupuis, Marie-Pierre Gaub, Raoul Herbrecht, Patrick Lutz, Susan Chan
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; Hôpital de
Hautepierre, Hôpitaux Universitaires de Stras-bourg, Avenue Molière, 67100 Strasbourg, France; Plate-forme Régionale INCa
de Génétique Moléculaire des Cancers d’Alsace, Avenue Mo-lière, 67098 Strasbourg, France
Received December 12, 2012; Accepted January 6, 2013; Epub January 17, 2013; Published January 25, 2013
Abstract: 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. (AJBR1212004).
Keywords: B cell leukemia, Ikaros, tumor suppressor
Address all correspondence to:
Drs. Philippe Kastner and Susan Chan
IGBMC, 1 rue Laurent Fries 67404 Illkirch Cedex, France.
Phone: 33-3-88 65 34 61; Fax: 33-3-88 65 32 01
E-mail: scpk@igbmc.fr
Review Article
Function of Ikaros as a tumor suppressor in B cell acute lympho-blastic
leukemia
Philippe Kastner, Arnaud Dupuis, Marie-Pierre Gaub, Raoul Herbrecht, Patrick Lutz, Susan Chan
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; Hôpital de
Hautepierre, Hôpitaux Universitaires de Stras-bourg, Avenue Molière, 67100 Strasbourg, France; Plate-forme Régionale INCa
de Génétique Moléculaire des Cancers d’Alsace, Avenue Mo-lière, 67098 Strasbourg, France
Received December 12, 2012; Accepted January 6, 2013; Epub January 17, 2013; Published January 25, 2013
Abstract: 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. (AJBR1212004).
Keywords: B cell leukemia, Ikaros, tumor suppressor
Address all correspondence to:
Drs. Philippe Kastner and Susan Chan
IGBMC, 1 rue Laurent Fries 67404 Illkirch Cedex, France.
Phone: 33-3-88 65 34 61; Fax: 33-3-88 65 32 01
E-mail: scpk@igbmc.fr
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