SQSTM1 and Paget's disease of bone

R. Layfield, Lynne Hocking

Research output: Contribution to journalEditorialpeer-review

59 Citations (Scopus)


Abstract. Mutations in the Sequestosome 1 gene (SQSTM1; also known as p62) have recently been identified as the cause of 5q35-linked Paget’s disease of bone (PDB). All of the mutations identified to date affect the ubiquitin-associated (UBA) domain of SQSTM1, a region of the protein that binds noncovalently to ubiquitin. In this review we consider the possible functional significance of the SQSTM1-ubiquitin interaction, and consequences of the SQSTM1 UBA domain mutations. Clarification of the in vivo roles of SQSTM1 in bone-cell function will be central to improving our understanding of the molecular pathogenesis of PDB and related conditions.
Original languageEnglish
Pages (from-to)347-357
Number of pages10
JournalCalcified Tissue International
Issue number5
Publication statusPublished - 2005


  • SQSTM1
  • p62
  • Paget’s disease of bone
  • Ubiquitin
  • UBA domain


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