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.
journal_name
Proc Natl Acad Sci U S Aauthors
Jones DC,Schweitzer MN,Wein M,Sigrist K,Takagi T,Ishii S,Glimcher LHdoi
10.1073/pnas.1003727107subject
Has Abstractpub_date
2010-05-04 00:00:00pages
8254-8issue
18eissn
0027-8424issn
1091-6490pii
1003727107journal_volume
107pub_type
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