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Article

Mutant p62P392L stimulation of osteoclast differentiation in Paget's disease of bone.

Endocrinology - 1 Nov 2011

Sundaram Kumaran, Shanmugarajan Srinivasan, Rao D Sudhaker, Reddy Sakamuri V

Abstract excerpt

Paget's disease of the bone (PDB) is an autosomal dominant trait with genetic heterogeneity, characterized by abnormal osteoclastogenesis. Sequestosome 1 (p62) is a scaffold protein that plays an important role in receptor activator of nuclear factor κB (RANK) signaling essential for osteoclast (OCL) differentiation. p62P392L mutation in the ubiquitin-associated (UBA) domain is widely associated with PDB;...

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Source

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Identifiers

PMID: 21878516
DOI: 10.1210/en.2011-1225
PMCID: PMC3198995

Genes

CYLD
TNF

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Mutant p62P392L stimulation of osteoclast differentiation in Paget's disease of bone.PMID 21878516Read ↗

The p62 P392L mutation linked to Paget's disease induces activation of human osteoclasts.Semantic proximity Read ↗

A Novel Mutation (K378X) in the <i>Sequestosome 1</i> Gene Associated With Increased NF-κB Signaling and Paget's Disease of Bone With a Severe PhenotypeSemantic proximity Read ↗

The adaptor protein p62/SQSTM1 in osteoclast signaling pathwaysSemantic proximity Read ↗

Deubiquitinating enzyme CYLD negatively regulates RANK signaling and osteoclastogenesis in miceSemantic proximity Read ↗

Mutation of the sequestosome 1 (p62) gene increases osteoclastogenesis but does not induce Paget disease.Semantic proximity Read ↗

Gene expression profile in osteoclasts from patients with Paget's disease of bone.Semantic proximity Read ↗

Involvement of kinase PKC-zeta in the p62/p62(P392L)-driven activation of NF-κB in human osteoclasts.Semantic proximity Read ↗

A SQSTM1/p62 mutation linked to Paget’s disease increases the osteoclastogenic potential of the bone microenvironmentSemantic proximity Read ↗

New insights into the role of sequestosome 1/p62 mutant proteins in the pathogenesis of Paget's disease of bone.Semantic proximity Read ↗

Paget's disease of bone and genetic disorders of RANKL/OPG/RANK/NF-kappaB signaling.Semantic proximity Read ↗

p62/sequestosome 1 deficiency accelerates osteoclastogenesis in vitro and leads to Paget’s disease–like bone phenotypes in miceSemantic proximity Read ↗

Alternative splicing in osteoclasts and Paget's disease of bone.Semantic proximity Read ↗

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