Ubiquitin-mediated signalling and Paget's disease of bone.
Ubiquitin-mediated signalling and Paget's disease of bone.
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DOI：
10.1186/1471-2091-8-s1-s5
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发表时间：
2007-11-22
期刊：
影响因子：
--
通讯作者：
Shaw B
中科院分区：
文献类型：
--
作者：
Layfield R;Shaw B
Multiple steps in the RANK-NF-κB signalling pathway are regulated by ubiquitylation. Mutations affecting different components of this pathway, including the ubiquitin binding p62 signalling adapter protein, are found in patients with Paget's disease of bone or related syndromes. Here, we review the molecular defects and potential disease mechanisms in these conditions and conclude that the mutations may confer a common increased sensitivity of osteoclasts to cytokines, resulting in disordered NF-κB-dependent osteoclast function. Modulation of the osteoclast RANK-NF-κB signalling axis may represent a viable therapeutic strategy for Paget's disease and other conditions where excessive bone resorption or remodelling is a feature. Publication history: Republished from Current BioData's Targeted Proteins database (TPdb; ).
