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

Research output: Contribution to journalArticle

90 Citations (Scopus)

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

Loss of Ubiquitin-Binding Associated With Paget's Disease of Bone p62 (SQSTM1) Mutations. / Cavey, J. R.; Ralston, S; Hocking, Lynne; Sheppard, P. W.; Ciani, B.; Searle, MS.; Layfield, R.

In: Journal of Bone and Mineral Research, Vol. 20, No. 4, 04.2005, p. 619-624.

Research output: Contribution to journalArticle

Cavey, JR, Ralston, S, Hocking, L, Sheppard, PW, 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, vol. 20, no. 4, pp. 619-624. https://doi.org/10.1359/JBMR.041205
Cavey, J. R. ; Ralston, S ; Hocking, Lynne ; Sheppard, P. W. ; Ciani, B. ; Searle, MS. ; Layfield, R. / Loss of Ubiquitin-Binding Associated With Paget's Disease of Bone p62 (SQSTM1) Mutations. In: Journal of Bone and Mineral Research. 2005 ; Vol. 20, No. 4. pp. 619-624.
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