Aberrant stabilization of c-Myc protein in some lymphoblastic leukemias

Leukemia. 2006 Sep;20(9):1572-81. doi: 10.1038/sj.leu.2404317. Epub 2006 Jul 20.

Abstract

Overexpression of the c-Myc oncoprotein is observed in a large number of hematopoietic malignancies, and transgenic animal models have revealed a potent role for c-Myc in the generation of leukemias and lymphomas. However, the reason for high c-Myc protein levels in most cases is unknown. We examined whether aberrant protein stabilization could be a mechanism of c-Myc overexpression in leukemia cell lines and in primary bone marrow samples from pediatric acute lymphoblastic leukemia (ALL) patients. We found that c-Myc protein half-life was prolonged in the majority of leukemia cell lines and bone marrow samples tested. There were no mutations in the c-myc gene in any of the leukemia cell lines that could account for increased c-Myc stability. However, abnormal phosphorylation at two conserved sites, Threonine 58 and Serine 62, was observed in leukemia cell lines with stabilized c-Myc. Moreover, stabilized c-Myc from the ALL cell lines showed decreased affinity for glycogen synthase kinase3beta, the kinase that phosphorylates c-Myc at Threonine 58 and facilitates its degradation. These findings reveal that deregulation of the c-Myc degradation pathway controlled by Serine 62 and Threonine 58 phosphorylation is a novel mechanism for increased expression of a potent oncoprotein known to be involved in hematopoietic malignancies.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Amino Acid Sequence
  • Blotting, Western
  • Cell Line
  • Child
  • Enzyme Activation
  • Gene Amplification
  • Glycogen Synthase Kinase 3 / metabolism
  • Glycogen Synthase Kinase 3 beta
  • Humans
  • Immunoprecipitation
  • In Situ Hybridization, Fluorescence
  • Molecular Sequence Data
  • Phosphatidylinositol 3-Kinases / metabolism
  • Phosphorylation
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / metabolism*
  • Proteasome Endopeptidase Complex / metabolism
  • Proto-Oncogene Proteins c-myc / chemistry
  • Proto-Oncogene Proteins c-myc / genetics
  • Proto-Oncogene Proteins c-myc / metabolism*
  • Sequence Homology, Amino Acid
  • Serine / metabolism
  • Threonine / metabolism

Substances

  • Proto-Oncogene Proteins c-myc
  • Threonine
  • Serine
  • Phosphatidylinositol 3-Kinases
  • Glycogen Synthase Kinase 3 beta
  • Glycogen Synthase Kinase 3
  • Proteasome Endopeptidase Complex
  • ATP dependent 26S protease