Characterisation and therapeutic intervention of two novel mouse models of Paget's disease of bone

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Paget?s disease (PDB) is a common bone disease that affects about 3% of people over the age of 55 in the UK which causes pain, softening of the bones, deafness and arthritis. Genetic factors are important in the disease and two abnormal genes have been discovered. It is unclear how these faulty genes cause PDB and it is also unclear if treatment might be able to protect against disease complications if given early enough. The aim of this project is to develop an experimental model for PDB in order to find out more about why the disease occurs and to determine if early treatment might be effective in preventing complications. If our experiments are successful the information gained from these studies will be used to help design clinical trials of preventative therapy in patients with the faulty genes.