Genetic resistance to JAK2 enzymatic inhibitors is overcome by HSP90 inhibition

J Exp Med. 2012 Feb 13;209(2):259-73. doi: 10.1084/jem.20111694. Epub 2012 Jan 23.

Abstract

Enzymatic inhibitors of Janus kinase 2 (JAK2) are in clinical development for the treatment of myeloproliferative neoplasms (MPNs), B cell acute lymphoblastic leukemia (B-ALL) with rearrangements of the cytokine receptor subunit cytokine receptor-like factor 2 (CRLF2), and other tumors with constitutive JAK2 signaling. In this study, we identify G935R, Y931C, and E864K mutations within the JAK2 kinase domain that confer resistance across a panel of JAK inhibitors, whether present in cis with JAK2 V617F (observed in MPNs) or JAK2 R683G (observed in B-ALL). G935R, Y931C, and E864K do not reduce the sensitivity of JAK2-dependent cells to inhibitors of heat shock protein 90 (HSP90), which promote the degradation of both wild-type and mutant JAK2. HSP90 inhibitors were 100-1,000-fold more potent against CRLF2-rearranged B-ALL cells, which correlated with JAK2 degradation and more extensive blockade of JAK2/STAT5, MAP kinase, and AKT signaling. In addition, the HSP90 inhibitor AUY922 prolonged survival of mice xenografted with primary human CRLF2-rearranged B-ALL further than an enzymatic JAK2 inhibitor. Thus, HSP90 is a promising therapeutic target in JAK2-driven cancers, including those with genetic resistance to JAK enzymatic inhibitors.

Publication types

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

MeSH terms

  • Animals
  • Cell Line, Tumor
  • Cell Proliferation
  • DNA Primers / genetics
  • Female
  • Flow Cytometry
  • Gene Expression Profiling
  • HSP90 Heat-Shock Proteins / antagonists & inhibitors*
  • HSP90 Heat-Shock Proteins / metabolism
  • Humans
  • Immunoblotting
  • Immunohistochemistry
  • Isoxazoles / pharmacology*
  • Isoxazoles / therapeutic use
  • Janus Kinase 2 / antagonists & inhibitors*
  • Janus Kinase 2 / genetics*
  • Janus Kinase 2 / metabolism
  • Leukemia, B-Cell / drug therapy
  • Leukemia, B-Cell / enzymology*
  • Leukemia, B-Cell / genetics
  • Luciferases
  • Mice
  • Mice, Inbred BALB C
  • Mutagenesis
  • Mutation, Missense / genetics
  • Myeloproliferative Disorders / drug therapy
  • Myeloproliferative Disorders / enzymology*
  • Myeloproliferative Disorders / genetics
  • Phosphorylation
  • RNA, Small Interfering / genetics
  • Receptors, Cytokine / genetics
  • Receptors, Cytokine / metabolism
  • Resorcinols / pharmacology*
  • Resorcinols / therapeutic use
  • Signal Transduction / physiology*
  • X-Ray Microtomography

Substances

  • 5-(2,4-dihydroxy-5-isopropylphenyl)-4-(4-morpholin-4-ylmethylphenyl)isoxazole-3-carboxylic acid ethylamide
  • CRLF2 protein, human
  • DNA Primers
  • HSP90 Heat-Shock Proteins
  • Isoxazoles
  • RNA, Small Interfering
  • Receptors, Cytokine
  • Resorcinols
  • Luciferases
  • Janus Kinase 2