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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98 Citations (Scopus)


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
Issue number4
Early online date6 Dec 2004
Publication statusPublished - Apr 2005


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


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