Quantitative interaction mapping reveals an extended UBX domain in ASPL that disrupts functional p97 hexamers

Nat Commun. 2016 Oct 20:7:13047. doi: 10.1038/ncomms13047.

Abstract

Interaction mapping is a powerful strategy to elucidate the biological function of protein assemblies and their regulators. Here, we report the generation of a quantitative interaction network, directly linking 14 human proteins to the AAA+ ATPase p97, an essential hexameric protein with multiple cellular functions. We show that the high-affinity interacting protein ASPL efficiently promotes p97 hexamer disassembly, resulting in the formation of stable p97:ASPL heterotetramers. High-resolution structural and biochemical studies indicate that an extended UBX domain (eUBX) in ASPL is critical for p97 hexamer disassembly and facilitates the assembly of p97:ASPL heterotetramers. This spontaneous process is accompanied by a reorientation of the D2 ATPase domain in p97 and a loss of its activity. Finally, we demonstrate that overproduction of ASPL disrupts p97 hexamer function in ERAD and that engineered eUBX polypeptides can induce cell death, providing a rationale for developing anti-cancer polypeptide inhibitors that may target p97 activity.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Brain / pathology
  • Cell Proliferation
  • Crystallography, X-Ray
  • Endoplasmic Reticulum-Associated Degradation / physiology*
  • HEK293 Cells
  • Humans
  • Intracellular Signaling Peptides and Proteins
  • Mutation
  • Oncogene Proteins, Fusion / chemistry
  • Oncogene Proteins, Fusion / isolation & purification
  • Oncogene Proteins, Fusion / metabolism*
  • Peptides / genetics
  • Peptides / metabolism
  • Protein Binding
  • Protein Domains / physiology*
  • Protein Engineering
  • Protein Interaction Maps
  • Protein Multimerization
  • Protein Structure, Quaternary
  • Recombinant Proteins / chemistry
  • Recombinant Proteins / isolation & purification
  • Recombinant Proteins / metabolism
  • Valosin Containing Protein / chemistry
  • Valosin Containing Protein / isolation & purification
  • Valosin Containing Protein / metabolism*

Substances

  • ASPSCR1 protein, human
  • Intracellular Signaling Peptides and Proteins
  • Oncogene Proteins, Fusion
  • Peptides
  • Recombinant Proteins
  • VCP protein, human
  • Valosin Containing Protein