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Volume 347:1622-1623 November 14, 2002 Number 20
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Osteoprotegerin Deficiency and Juvenile Paget's Disease

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 by Whyte, M. P.
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To the Editor: Whyte et al. (July 18 issue)1 report that homozygous deletion of the gene encoding osteoprotegerin is a potential cause of juvenile Paget's disease in some Navajo patients. A deficiency of osteoprotegerin leads to unopposed effects of its ligand, receptor activator of nuclear factor {kappa}B (RANK) ligand, resulting in enhanced osteoclastic bone resorption and profound bone loss. Since osteoprotegerin also serves as a receptor antagonist for tumor necrosis factor–related apoptosis-inducing ligand (TRAIL),2 one cannot rule out the possibility that excessive TRAIL effects contributed to the skeletal phenotype.

Increased susceptibility to infectious and cardiovascular diseases in patients with juvenile . . . [Full Text of this Article]




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