Loss of Ubiquitin-Binding Associated With Paget's Disease of Bone p62 (SQSTM1) Mutations

J. R. Cavey, S Ralston, Lynne Hocking, P. W. Sheppard, B. Ciani, MS. Searle, R. Layfield

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Abstract

We have studied the effects of various PDB-causing mutations of SQSTMl on the in vitro ubiquitin-binding properties of the p62 protein. All mutations caused loss of monoubiquitin-binding and impaired K48-linked polyubiquitin-binding, which was only evident at physiological temperature. This suggests that SQSTMI mutations predispose to PDB through a common mechanism that depends on loss of ubiquitin-binding by p62.

Original languageEnglish
Pages (from-to)619-624
Number of pages5
JournalJournal of Bone and Mineral Research
Volume20
Issue number4
Early online date6 Dec 2004
DOIs
Publication statusPublished - Apr 2005

Keywords

  • SQSTM1
  • p62
  • ubiquitin
  • Paget's disease of bone
  • ubiquitin-associated domain
  • familial expansile osteolysis
  • interacting protein P62
  • UBA domain
  • gene
  • TNFRSF11A
  • rank
  • osteoclastogenesis
  • duplication
  • pathway

Cite this

Cavey, J. R., Ralston, S., Hocking, L., Sheppard, P. W., Ciani, B., Searle, MS., & Layfield, R. (2005). Loss of Ubiquitin-Binding Associated With Paget's Disease of Bone p62 (SQSTM1) Mutations. Journal of Bone and Mineral Research, 20(4), 619-624. https://doi.org/10.1359/JBMR.041205