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- W2024448924 abstract "Abstract: Identification of the RANKL/OPG/RANK/NF‐kB (receptor activator of nuclear factor κ‐B ligand / osteoprotegerin) signaling pathway as the major regulatory system for osteoclastogenesis began with discovery of these ligands and receptors in the tumor necrosis factor (TNF) superfamily. Subsequently, genetically altered mice revealed physiologic roles for these proteins in bone biology. However, full appreciation of their significance for the human skeleton came from clinical characterization of several extremely rare, heritable disorders followed by discovery of their genetic bases. Familial expansile osteolysis (FEO) is an autosomal dominant disorder featuring constitutive activation of RANK due to an 18‐bp tandem duplication in its gene ( TNFRSF11A ). A similar, 27‐bp duplication causes what has been called a familial form of early‐onset Paget's disease of bone (PDB2). Expansile skeletal hyperphosphatasia (ESH) is allelic to FEO and PDB2 and involves a 15‐bp tandem duplication in TNFRSF11A . Autosomal recessive inheritance of deactivating mutations of the gene encoding OPG ( TNFRSF11B ) causes most cases of juvenile Paget disease. These disorders feature high bone turnover, deafness during early childhood, “idiopathic external lysis” of adult teeth, and sometimes focal lesions in appendicular bones that mimic active PDB. Biochemical markers indicate rapid skeletal remodeling. In FEO, osteolysis progresses to fat‐filled bone rather than to osteosclerosis. Antiresorptive therapy with bisphosphonates can be effective for each disorder." @default.
- W2024448924 created "2016-06-24" @default.
- W2024448924 creator A5036775753 @default.
- W2024448924 date "2006-04-01" @default.
- W2024448924 modified "2023-10-10" @default.
- W2024448924 title "Paget's Disease of Bone and Genetic Disorders of RANKL/OPG/RANK/NF‐κB Signaling" @default.
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- W2024448924 doi "https://doi.org/10.1196/annals.1346.016" @default.
- W2024448924 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/16831914" @default.
- W2024448924 hasPublicationYear "2006" @default.
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