Structure of a Janus kinase cytokine receptor complex reveals the basis for dimeric activation

Science. 2022 Apr 8;376(6589):163-169. doi: 10.1126/science.abn8933. Epub 2022 Mar 10.

Abstract

Cytokines signal through cell surface receptor dimers to initiate activation of intracellular Janus kinases (JAKs). We report the 3.6-angstrom-resolution cryo-electron microscopy structure of full-length JAK1 complexed with a cytokine receptor intracellular domain Box1 and Box2 regions captured as an activated homodimer bearing the valine→phenylalanine (VF) mutation prevalent in myeloproliferative neoplasms. The seven domains of JAK1 form an extended structural unit, the dimerization of which is mediated by close-packing of the pseudokinase (PK) domains from the monomeric subunits. The oncogenic VF mutation lies within the core of the JAK1 PK interdimer interface, enhancing packing complementarity to facilitate ligand-independent activation. The carboxy-terminal tyrosine kinase domains are poised for transactivation and to phosphorylate the receptor STAT (signal transducer and activator of transcription)-recruiting motifs projecting from the overhanging FERM (four-point-one, ezrin, radixin, moesin)-SH2 (Src homology 2)-domains. Mapping of constitutively active JAK mutants supports a two-step allosteric activation mechanism and reveals opportunities for selective therapeutic targeting of oncogenic JAK signaling.

MeSH terms

  • Allosteric Regulation
  • Cryoelectron Microscopy
  • Enzyme Activation
  • Humans
  • Janus Kinase 1* / chemistry
  • Janus Kinase 1* / metabolism
  • Mutation
  • Myeloproliferative Disorders / enzymology
  • Myeloproliferative Disorders / genetics
  • Phosphorylation
  • Protein Multimerization
  • Receptors, Cytokine* / chemistry
  • STAT Transcription Factors / metabolism
  • src Homology Domains*

Substances

  • Receptors, Cytokine
  • STAT Transcription Factors
  • Janus Kinase 1