Article
Zoledronic acid prevents pagetic-like lesions and accelerated bone loss in the p62P394L mouse model of Paget's disease.
Disease models & mechanisms - 23 Aug 2018
Daroszewska Anna, Rose Lorraine, Sarsam Nadine, Charlesworth Gemma, Prior Amanda, Rose Kenneth, Ralston Stuart H, van 't Hof Robert J
Abstract excerpt
Paget's disease of bone (PDB) is an age-related metabolic bone disorder, characterised by focally increased and disorganised bone remodelling initiated by abnormal and hyperactive osteoclasts. The germline P392L mutation of SQSTM1 (encoding p62) is a strong genetic risk factor for PDB in humans, and the equivalent mutation in mice (P394L) causes a PDB-like disorder. However, it is unclear why pagetic lesions...
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