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Paget disease of bone
G. David Roodman, Jolene J. Windle
G. David Roodman, Jolene J. Windle
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Science in Medicine

Paget disease of bone

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Abstract

Paget disease of bone (PD) is characterized by excessive bone resorption in focal areas followed by abundant new bone formation, with eventual replacement of the normal bone marrow by vascular and fibrous tissue. The etiology of PD is not well understood, but one PD-linked gene and several other susceptibility loci have been identified, and paramyxoviral gene products have been detected in pagetic osteoclasts. In this review, the pathophysiology of PD and evidence for both a genetic and a viral etiology for PD will be discussed.

Authors

G. David Roodman, Jolene J. Windle

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Figure 4

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Structure of the p62 protein. The blocks indicate domains that mediate a...
Structure of the p62 protein. The blocks indicate domains that mediate association with other proteins or are hypothesized to mediate these associations based upon homology with other proteins. The solid lines below the protein indicate stretches of sequence identity (of 20 amino acids or more) among the mouse, rat, and human p62 proteins. The arrows above the protein indicate the Paget disease-associated mutations identified to date. The splice donor and stop mutations result in a truncated protein lacking the UBA domain. PEST denotes hydrophobic regions that target proteins for rapid degradation (P, proline; E, glutaric acid; S, serine; T, threonine).

Copyright © 2026 American Society for Clinical Investigation
ISSN: 0021-9738 (print), 1558-8238 (online)

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