期刊
JOURNAL OF CRANIOFACIAL SURGERY
卷 23, 期 1, 页码 61-66出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/SCS.0b013e318240c8c4
关键词
Runx2; membranous bone; Sox9; endochondral bone
类别
资金
- NIH/NIDCR [R21 DE0177711, RO1 DE01607]
- UC [07-10677]
- Thomas R. Bales Endowed Chair
Nell-1, first identified by its overexpression in synostotic cranial sutures, is a novel osteoinductive growth and differentiation factor. To further define Nell-1's role in craniofacial patterning, we characterized defects of the ENU-induced Nell-1-deficient (END) mice, focusing on both intramembranous and endochondral cranial bones. Results showed that calvarial bones of neonatal END mice were reduced in thickness and density, with a phenotype resembling calvarial cleidocraniodysplasia. In addition, a global reduction in osteoblast markers was observed, including reductions in Runx2, alkaline phosphatase, and osteocalcin. Remarkably, detailed analysis of endochondral bones showed dysplasia as well. The chondrocranium in the END mouse showed enrichment for early, proliferating Sox9(+) chondrocytes, whereas in contrast markers of chondrocytes maturation were reduced. These data suggest that Nell-1 is an important growth factor for regulation of osteochondral differentiation, by regulating both Runx2 and Sox9 expression within the calvarium. In summary, Nell-1 is required for normal craniofacial membranous and endochondral skeletal development.
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