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Growth of cranial synchondroses and sutures requires polycystin-1
Elona Kolpakova-Hart1, Brandeis McBratney-Owen, Bo Hou
1Department of Developmental Biology, Harvard School of Dental Medicine, Boston, Massachusetts 02115, USA.
Insights
Polycystin-1 (Pkd1) is crucial for skull base and craniofacial suture development in mice. Pkd1 deficiency causes premature closure of skull base synchondroses and impaired suture growth, impacting cranial bone development.
Area of Science:
- Developmental Biology
- Genetics
- Orthopedics
Background:
- Coordinated craniofacial and skull base growth is vital for vertebrate development.
- Understanding molecules regulating skull growth is key for diagnosing and treating craniofacial defects.
Purpose of the Study:
- To investigate the role of polycystin-1 (Pkd1) in craniofacial bone and skull base development.
- To identify Pkd1's function in osteochondroprogenitor cell proliferation and signaling pathways.
Main Methods:
- Generation and analysis of Pkd1-deficient mice.
- Histological examination of skull base synchondroses and craniofacial sutures.
- Assessment of cell proliferation and signaling pathway activation (Erk1/2).
Main Results:
- Pkd1 deficiency led to premature closure of presphenoid and sphenooccipital synchondroses.
- Mice lacking Pkd1 in neural crest cells showed impaired postnatal growth at craniofacial suture osteogenic fronts.
- Up-regulation of the Erk1/2 signaling pathway was observed in Pkd1-deficient skeletal tissues.
Conclusions:
- Polycystin-1 is essential for the proliferation of cranial osteochondroprogenitor cells from both mesodermal and neural crest origins.
- Pkd1 plays a critical role in regulating skull growth and suture development.
- Dysregulation of Pkd1 impacts cranial base and suture development, potentially through the Erk1/2 pathway.
Abstract:
In vertebrates, coordinated embryonic and postnatal growth of the craniofacial bones and the skull base is essential during the expansion of the rostrum and the brain. Identification of molecules that regulate skull growth is important for understanding the nature of craniofacial defects and for development of non-invasive biologically based diagnostics and therapies. Here we report on spatially restricted growth defects at the skull base and in craniofacial sutures of mice deficient for polycystin-1 (Pkd1). Mutant animals reveal a premature closure of both presphenoid and sphenooccipital synchondroses at the cranial base. Furthermore, knockout mice lacking Pkd1 in neural crest cells are characterized by impaired postnatal growth at the osteogenic fronts in craniofacial sutures that are subjected to tensile forces. Our data suggest that polycystin-1 is required for proliferation of subpopulations of cranial osteochondroprogenitor cells of both mesodermal and neural crest origin during skull growth. However, the Erk1/2 signalling pathway is up-regulated in the Pkd1-deficient skeletal tissue, similarly to that previously reported for polycystic kidney.
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