Non-Functional ("haploinsufficient"), but Not Dominant Negative Clonal IKZF1Deletions Confer an Adverse Prognosis in Adult BCR-ABL-Negative Acute Lymphoblastic Leukemia

Autor: Kobitzsch, Benjamin, Gökbuget, Nicola, Schwartz, Stefan, Reinhardt, Richard, Brueggemann, Monika, Viardot, Andreas, Wäsch, Ralph, Starck, Michael, Thiel, Eckhard, Hoelzer, Dieter, Burmeister, Thomas
Zdroj: Blood; December 2015, Vol. 126 Issue: 23 p2617-2617, 1p
Abstrakt: Background:IKAROS (IKZF1) is a key transcription factor for B-cell development. The IKZF1 protein exerts its functions as a dimer (homodimer or heterodimer with other IKAROS family members). Various IKZF1intragenic deletions have been identified as recurrent aberrations in acute lymphoblastic leukemia: those resulting in the loss of transcription of one allele or at least loss of the dimerization domain ("haploinsufficient", "non-functional") and those involving only the DNA-binding domain but retaining the dimerization domain of the transcription factor, leading to so-called "dominant negative" isoforms.
Databáze: Supplemental Index