Includes bibliographical references and index.

Online resource; title from PDF title page (ScienceDirect, viewed May 10, 2016).

Front Cover; Advances in Pathobiology and Management of Paget's Disease of Bone; Copyright Page; Contents; List of Contributors; About the Editor; Preface; 1 Clinical Perspectives of Paget's Disease of Bone; Introduction; Natural History; Clinical Features and Complications; Diagnosis; Pathology; Biochemistry; Epidemiology; Secular Change in Paget's Disease; References; 2 Viral Etiology of Paget's Disease of Bone; Introduction; Paramyxo-Viral Etiology; Viral and Genetic Interactions; Acknowledgments; References; 3 Genetics of Paget's Disease of Bone; Introduction

Importance of Genetic FactorsSQSTM1; TNFRSF11B; VCP; Chromosome 1p13 Locus; Chromosome 7q33; Chromosome 8q24; Chromosome 10p13; Chromosome 14q32; Chromosome 15q24; Chromosome 18q21; Genetic Determinants of Disease Severity; Clinical Implications; References; 4 Developmental Aspects of Pagetic Osteoclasts; Introduction; Increased Sensitivity of OCL Progenitors to Osteotropic Factors; Increased Sensitivity to RANKL and TNFα; Increased Sensitivity to 1,25-(OH)2D3; Increased Nuclei/OCL; Increased Expression of IL-6; MVNP Activation of TBK1 Increases IL-6, OCL Formation, and Nuclei/OCL

MVNP Downregulation of FoxO3 and Sirt1 also Increase IL-6Increased Irregular Woven Bone Formation Induced by Pagetic OCL; EphrinB2/EphB4 Signaling; IGF1, FGF2, and CXCL5; Conclusion; Disclosures; References; 5 Mutant SQSTM1/p62 Signaling in Paget's Disease of Bone; Introduction; RANKL-Induced NFκB Signaling and Osteoclastogenesis; Defective NFκB Signaling Associated With Mutant SQSTM1/p62; SQSTM1/p62-Mediated RANKL-Induced AP-1, NFAT, and ERK Signaling; SQSTM1/p62-Mediated Regulation of Keap1/Nrf2 Signaling; SQSTM1/p62-Mediated Apoptosis; SQSTM1/p62-Mediated Autophagy

SQSTM1/p62 as a Signaling Hub Orchestrating the Interplay Between NFκB, Keap1 and AutophagyReferences; 6 Early Onset Pagetic Disorders: Implications for Late-Onset Paget's Disease of Bone; Introduction; Early-Onset Pagetic Syndromes; RANK; Juvenile Paget's Disease; Osteoprotegerin; Multisystem Proteinopathy; Valosin-containing protein; Molecular Mechanisms Leading to Early-Onset Pagetic Disorders and Relevance for Late-onset PDB; Classic PDB: Association With Genes Involved in RANK Signaling and/or Osteoclast Formation; Classic PDB: Genes Involved in Protein Degradation Pathways

PDB: A Disorder of Protein Conformation?Acknowledgments; References; 7 Osteosarcoma in Paget's Disease of Bone; Introduction; Epidemiology; Clinical Presentation; Histopathology; Treatment and Prognosis; The Progression From Normal Bone to Paget's Disease to Pagetic Osteosarcoma; Genes Differentially Expressed in Paget's Disease and Pagetic Osteosarcoma; Acknowledgments; References; 8 Paget's Disease of Bone: Prognosis and Complications; Introduction; Prognosis of Paget's Disease of Bone; Morbidity and Quality of Life; Mortality; Impact of Pharmacological Treatment of PDB on the Prognosis

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