Pre-BCR signaling in precursor B-cell acute lymphoblastic leukemia regulates PI3K/AKT, FOXO1 and MYC, and can be targeted by SYK inhibition

Leukemia. 2016 Jun;30(6):1246-54. doi: 10.1038/leu.2016.9. Epub 2016 Feb 5.

Abstract

Precursor-B-cell receptor (pre-BCR) signaling and spleen tyrosine kinase (SYK) recently were introduced as therapeutic targets for patients with B-cell acute lymphoblastic leukemia (B-ALL), but the importance of this pathway in B-ALL subsets and mechanism of downstream signaling have not fully been elucidated. Here, we provide new detailed insight into the mechanism of pre-BCR signaling in B-ALL. We compared the effects of pharmacological and genetic disruption of pre-BCR signaling in vitro and in mouse models for B-ALL, demonstrating exquisite dependency of pre-BCR(+) B-ALL, but not other B-ALL subsets, on this signaling pathway. We demonstrate that SYK, PI3K/AKT, FOXO1 and MYC are important downstream mediators of pre-BCR signaling in B-ALL. Furthermore, we define a characteristic immune phenotype and gene expression signature of pre-BCR(+) ALL to distinguish them from other B-ALL subsets. These data provide comprehensive new insight into pre-BCR signaling in B-ALL and corroborate pre-BCR signaling and SYK as promising new therapeutic targets in pre-BCR(+) B-ALL.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cell Line
  • Forkhead Box Protein O1 / metabolism
  • Heterografts
  • Humans
  • Mice
  • Phosphatidylinositol 3-Kinases / metabolism
  • Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / metabolism*
  • Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / pathology
  • Precursor Cells, B-Lymphoid / chemistry*
  • Proto-Oncogene Proteins c-myc / metabolism
  • Receptors, Antigen, B-Cell / metabolism*
  • Signal Transduction*
  • Syk Kinase / metabolism

Substances

  • FOXO1 protein, human
  • Forkhead Box Protein O1
  • MYC protein, human
  • Proto-Oncogene Proteins c-myc
  • Receptors, Antigen, B-Cell
  • Phosphatidylinositol 3-Kinases
  • SYK protein, human
  • Syk Kinase