KRAS insertion mutations are oncogenic and exhibit distinct functional properties

Nat Commun. 2016 Feb 8:7:10647. doi: 10.1038/ncomms10647.

Abstract

Oncogenic KRAS mutations introduce discrete amino acid substitutions that reduce intrinsic Ras GTPase activity and confer resistance to GTPase-activating proteins (GAPs). Here we discover a partial duplication of the switch 2 domain of K-Ras encoding a tandem repeat of amino acids G60_A66dup in a child with an atypical myeloproliferative neoplasm. K-Ras proteins containing this tandem duplication or a similar five amino acid E62_A66dup mutation identified in lung and colon cancers transform the growth of primary myeloid progenitors and of Ba/F3 cells. Recombinant K-Ras(G60_A66dup) and K-Ras(E62_A66dup) proteins display reduced intrinsic GTP hydrolysis rates, accumulate in the GTP-bound conformation and are resistant to GAP-mediated GTP hydrolysis. Remarkably, K-Ras proteins with switch 2 insertions are impaired for PI3 kinase binding and Akt activation, and are hypersensitive to MEK inhibition. These studies illuminate a new class of oncogenic KRAS mutations and reveal unexpected plasticity in oncogenic Ras proteins that has diagnostic and therapeutic implications.

Publication types

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

MeSH terms

  • Animals
  • Child, Preschool
  • GTPase-Activating Proteins / metabolism*
  • Hepatocytes / metabolism*
  • Humans
  • Leukemia, Myelomonocytic, Juvenile / genetics*
  • Mice
  • Mutagenesis, Insertional*
  • Mutation
  • Phosphatidylinositol 3-Kinases / metabolism*
  • Proto-Oncogene Proteins c-akt / metabolism*
  • Proto-Oncogene Proteins p21(ras) / genetics*
  • Tandem Repeat Sequences
  • Tumor Stem Cell Assay

Substances

  • GTPase-Activating Proteins
  • KRAS protein, human
  • Phosphatidylinositol 3-Kinases
  • Proto-Oncogene Proteins c-akt
  • Proto-Oncogene Proteins p21(ras)