OTT-MAL is a deregulated activator of serum response factor-dependent gene expression

Mol Cell Biol. 2008 Oct;28(20):6171-81. doi: 10.1128/MCB.00303-08. Epub 2008 Aug 18.

Abstract

The OTT-MAL/RBM15-MKL1 fusion protein is the result of the recurrent translocation t(1;22) in acute megakaryocytic leukemia in infants. How it contributes to the malignancy is unknown. The 3' fusion partner, MAL/MKL1/MRTF-A, is a transcriptional coactivator of serum response factor (SRF). MAL plays a key role in regulated gene expression depending on Rho family GTPases and G-actin. Here we demonstrate that OTT-MAL is a constitutive activator of SRF and target gene expression. This requires the SRF-binding motif and the MAL-derived transactivation domain. OTT-MAL localizes to the nucleus and is not regulated by upstream signaling. OTT-MAL deregulation reflects its independence from control by G-actin, which fails to interact with OTT-MAL in coimmunoprecipitation experiments. Regulation cannot be restored by reintroduction of the entire MAL N terminus into the fusion protein. OTT-MAL also caused a delayed induction of the MAL-independent, ternary complex factor-dependent target genes c-fos and egr-1 and the mitogen-activated protein kinase/Erk pathway. With testing in heterologous tissue culture systems, however, we observed considerable antiproliferative effects of OTT-MAL. Our data suggest that the deregulated activation of MAL-dependent and -independent promoters results in tissue-specific functions of OTT-MAL.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Actins / metabolism
  • Animals
  • Early Growth Response Protein 1 / genetics
  • Extracellular Signal-Regulated MAP Kinases / metabolism
  • Gene Expression Regulation*
  • HeLa Cells
  • Humans
  • Intracellular Space / metabolism
  • Mice
  • NIH 3T3 Cells
  • Oncogene Proteins, Fusion / chemistry
  • Oncogene Proteins, Fusion / metabolism*
  • Phosphorylation
  • Protein Binding
  • Protein Structure, Tertiary
  • Protein Transport
  • Proto-Oncogene Proteins c-fos / metabolism
  • Serum Response Factor / metabolism*
  • Signal Transduction
  • Ternary Complex Factors / metabolism
  • rhoA GTP-Binding Protein / metabolism

Substances

  • Actins
  • Early Growth Response Protein 1
  • OTT-MAL fusion protein, human
  • Oncogene Proteins, Fusion
  • Proto-Oncogene Proteins c-fos
  • Serum Response Factor
  • Ternary Complex Factors
  • Extracellular Signal-Regulated MAP Kinases
  • rhoA GTP-Binding Protein