A quest for clarity in bone erosion: The role of sequestosome 1 in Paget's disease of bone

Alterations in the SQSTM1 gene are a putative cause of Paget’s disease of bone, yet results are conflicting about how these mutations impact osteoclasts, the cell type believed to be the main pathological contributor. In this issue of JBC, Zach et al. provide important new evidence that the protein...

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Bibliographic Details
Published in:The Journal of biological chemistry 2018-06, Vol.293 (24), p.9542-9543
Main Authors: Michalski, Megan N., Williams, Bart O.
Format: Article
Language:English
Subjects:
Animals
Cell Differentiation
Editors' Picks Highlights
Gene Deletion
Humans
Mice
Mutation
Osteitis Deformans - genetics
Osteitis Deformans - pathology
Osteoclasts - cytology
Osteoclasts - metabolism
Osteoclasts - pathology
Phenotype
Sequestosome-1 Protein - genetics
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Summary:Alterations in the SQSTM1 gene are a putative cause of Paget’s disease of bone, yet results are conflicting about how these mutations impact osteoclasts, the cell type believed to be the main pathological contributor. In this issue of JBC, Zach et al. provide important new evidence that the protein encoded by SQSTM1, p62, negatively regulates osteoclastogenesis and demonstrate that aged p62–deficient mice develop bone phenotypes similar to those of Paget’s disease. These findings help to clarify the role of this important protein and present new opportunities to interrogate bone biology.
ISSN:0021-9258
1083-351X
DOI:10.1074/jbc.H118.003689