Genetic inactivation of Ikaros is a rare event in human T-ALL.
The Ikaros (Ikzf1) gene, encoding a transcription regulator, is a major tumor suppressor in B-cell acute lymphoblastic leukemia (B-ALL). In the mouse, however, loss of Ikaros is primarily associated with T-ALL development. Whether Ikaros is also implicated in human T-ALL remains unclear. We studied Ikaros in 25 human T-ALL samples from diverse molecular subtypes at the mRNA, protein, sequence and genomic copy number level. We found that Ikaros was abnormal in only one sample: one allele was lost by genomic deletion, while proteins generated from the remaining allele were delocalized and concentrated at a single cytoplasmic structure. Thus, inactivation of Ikaros by deletion or mutation is rare in human T-ALL.
Department of Cancer Biology, Institut de Génétique et de Biologie Moléculaire et Cellulaire, Illkirch, France.
Ambroise
A
Marçais
Robin
R
Jeannet
Lucie
L
Hernandez
Jean
J
Soulier
François
F
Sigaux
Susan
S
Chan
Philippe
P
Kastner
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D055028:Comparative Genomic Hybridization; D017353:Gene Deletion; D015973:Gene Expression Regulation, Leukemic; D020868:Gene Silencing / Q000502:physiology; D006801:Humans; D051740:Ikaros Transcription Factor / Q000235:genetics* / Q000502:physiology; D007223:Infant; D019169:Jurkat Cells; D008875:Middle Aged; D009154:Mutation / Q000502:physiology; D054218:Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / Q000235:genetics*; D020033:Protein Isoforms / Q000235:genetics; D055815:Young Adult
C497239:IKZF1 protein, human; D020033:Protein Isoforms; D051740:Ikaros Transcription Factor