Cited by 21Bone Metabolism and DiseasesOsteoarthritis Treatment and MechanismsBone Tumor Diagnosis and Treatments

Uncoupling of growth plate maturation and bone formation in mice lacking both Schnurri-2 and Schnurri-3

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Jones, Dallas C., Schweitzer, Michelle N., Wein, Marc, Sigrist, Kirsten, Takagi, Tsuyoshi, Ishii, Shunsuke, Glimcher, Laurie H.·2010·Proceedings of the National Academy of Sciences

Abstract

Formation and remodeling of the skeleton relies on precise temporal and spatial regulation of genes expressed in cartilage and bone cells. Debilitating diseases of the skeletal system occur when mutations arise that disrupt these intricate genetic regulatory programs. Here, we report that mice bearing parallel null mutations in the adapter proteins Schnurri2 (Shn2) and Schnurri3 (Shn3) exhibit defects in patterning of the axial skeleton during embryogenesis. Postnatally, these compound mutant mice develop a unique osteochondrodysplasia. The deletion of Shn2 and Shn3 impairs growth plate maturation during endochondral ossification but simultaneously results in massively elevated trabecular bone formation. Hence, growth plate maturation and bone formation can be uncoupled under certain circumstances. These unexpected findings demonstrate that both unique and redundant functions reside in the Schnurri protein family that are required for proper skeletal patterning and remodeling.

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Authors
Jones, D. C., Schweitzer, M. N., Wein, M., Sigrist, K., Takagi, T., Ishii, S., & Glimcher, L. H.
Affiliations
  • Harvard University (US)
  • RIKEN (JP)
Venue
Proceedings of the National Academy of Sciences
Published
2010-04-19
Volume
107
Issue
18
Pages
8254-8258
ISSN
  • 0027-8424 (ISSN-L)
  • 1091-6490
OpenAlex
W2127745370
Cited by
21
References
28
Citations by year
2024: 1 · 2023: 2 · 2022: 1 · 2019: 2 · 2018: 1 · 2016: 1 · 2015: 1 · 2014: 1 · 2013: 4
FWCI
1.43
Type
journal article
Language
en
Open access
Locations
  • Proceedings of the National Academy of Sciences (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • National Institutes of Health
  • National Institute of Arthritis and Musculoskeletal and Skin Diseases (K99AR055668)
  • Eunice Kennedy Shriver National Institute of Child Health and Human Development (R01HD055601, HD055601)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Bone Metabolism and Diseases
  • Health Sciences › Medicine › Rheumatology › Osteoarthritis Treatment and Mechanisms
  • Health Sciences › Medicine › Rheumatology › Bone Tumor Diagnosis and Treatments
Keywords
adaptor proteins, endochondral ossification, trabecular bone formation, skeletal dysplasia, skeletal development, skeletal remodeling, compound mutant mice, gene redundancy
MeSH terms
Animals, DNA-Binding Proteins, Growth Plate, Osteochondrodysplasias, Osteogenesis, Phenotype, Bone Density, Mice, Knockout, Gene Expression Regulation, Developmental, Gene Dosage, Mice

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