Osteoprotegerin Deficiency and Juvenile Paget's Disease
Top Cited Papers
- 18 July 2002
- journal article
- case report
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 347 (3) , 175-184
- https://doi.org/10.1056/nejmoa013096
Abstract
Juvenile Paget's disease, an autosomal recessive osteopathy, is characterized by rapidly remodeling woven bone, osteopenia, fractures, and progressive skeletal deformity. The molecular basis is not known. Osteoprotegerin deficiency could explain juvenile Paget's disease because osteoprotegerin suppresses bone turnover by functioning as a decoy receptor for osteoclast differentiation factor (also called RANK ligand).Keywords
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