research-article
Recent advances in understanding the molecular basis of Paget's disease of bone
University of Nottingham, United Kingdom
Correspondence to: Rob Layfield, University of Nottingham, School of Biomedical Sciences, University of Nottingham, Nottingham, NG7 2UH, United Kingdom; robert.layfield{at}nottingham.ac.uk
Accepted October 13, 2009
Paget’s disease of bone (PDB) is a relatively common disorder characterised by increased bone turnover within discrete lesions throughout the skeleton. The condition has a strong genetic component, with mutations affecting the SQSTM1 gene which encodes the p62 protein often found in PDB patients, although environmental factors also play an important role in disease aetiology. The precise disease mechanism(s) both in familial and sporadic forms of PDB is unclear, although defective RANK-NF-kB signalling has been suggested to contribute to the increased activity of pagetic osteoclasts in the former. Here, we review recent advances in the understanding of the molecular basis of PDB with particular emphasis on findings since 2008, and focus on newly defined functions of the p62 protein upon which SQSTM1 mutations may impact in the development of the pagetic phenotype.
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