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Article

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

1 Apr 2005

Abstract excerpt

UNLABELLED: We have studied the effects of various PDB-causing mutations of SQSTM1 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 SQSTM1 mutations predispose to PDB through a common mechanism that depends on loss of...

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PMID: 15765181
DOI: 10.1359/jbmr.041205

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Loss of Ubiquitin-Binding Associated With Paget's Disease of Bone p62 (<i>SQSTM1</i>) MutationsPMID 15765181Read ↗

Loss of ubiquitin binding is a unifying mechanism by which mutations of SQSTM1 cause Paget's disease of bone.Semantic proximity Read ↗

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Impact of p62/SQSTM1 UBA domain mutations linked to Paget's disease of bone on ubiquitin recognition.Semantic proximity Read ↗

Structural and functional studies of mutations affecting the UBA domain of SQSTM1 (p62) which cause Paget's disease of bone.Semantic proximity Read ↗

Characterization of a non-UBA domain missense mutation of sequestosome 1 (SQSTM1) in Paget's disease of bone.Semantic proximity Read ↗

Mutant p62/SQSTM1 UBA domains linked to Paget's disease of bone differ in their abilities to function as stabilization signals.Semantic proximity Read ↗

Three novel mutations in SQSTM1 identified in familial Paget's disease of bone.Semantic proximity Read ↗

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