Loss of ubiquitin-binding associated with Paget's disease of bone p62 (SQSTM1) mutations

被引：95

作者：

Cavey, JR

Ralston, SH

Hocking, LJ

Sheppard, PW

Ciani, B

Searle, MS

Layfield, R ^{[1
]}

机构：

[1] Univ Nottingham, Sch Med, Sch Biomed Sci, Nottingham NG7 2UH, England

[2] Univ Aberdeen, Dept Med & Therapeut, Aberdeen, Scotland

[3] BIOMOL Int LP, Exeter, Devon, England

[4] Univ Nottingham, Ctr Biomol Sci, Sch Chem, Nottingham NG7 2RD, England

来源：

JOURNAL OF BONE AND MINERAL RESEARCH | 2005年 / 20卷 / 04期

关键词：

SQSTM1; p62; ubiquitin; Paget's disease of bone; ubiquitin-associated domain;

D O I：

10.1359/JBMR.041205

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

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收藏

页码：619 / 624

页数：6

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