Disease relevance of Cranial Sutures

• Since noggin misexpression prevents cranial suture fusion in vitro and in vivo, we suggest that syndromic fgfr-mediated craniosynostoses may be the result of inappropriate downregulation of noggin expression [1].
• Saethre-Chotzen syndrome (SCS) is a human autosomal dominant disorder characterized by premature fusion of cranial sutures caused by mutations of the Twist gene encoding a basic helix-loop-helix (bHLH) transcription factor [2].
• Surgical correction of unilateral coronal synostosis offers a unique opportunity to examine the molecular differences between an abnormal and a normal cranial suture [3].
• However, as a group they had elevated total serum T3 concentrations, blunted TSH response to TRH, and accelerated closure of cranial sutures, all of which suggested subtle hyperthyroidism [4].
• This work supports genetic studies and yet shows that patients with Crouzon's disease have low FGF receptor 2 activity in cranial sutures [5].

Psychiatry related information on Cranial Sutures

• CONCLUSIONS: This series questions further the uncertain genetic determinism of craniosynostosis and seems to rule out the hypothesis of a deformation sequence following primary closure of the cranial sutures [6].

High impact information on Cranial Sutures

• The BMP antagonist noggin regulates cranial suture fusion [1].
• Previously, we reported NELL-1 as a novel molecule overexpressed during premature cranial suture closure in patients with craniosynostosis (CS), one of the most common congenital craniofacial deformities [7].
• Fgfr2c(C342Y/)(+) heterozygote mice are viable and fertile with shortened face, protruding eyes, premature fusion of cranial sutures, and enhanced Spp1 expression in the calvaria [8].
• Over-expression of Nell1 in the developing cranial sutures in both human and mouse induces craniosynostosis, the premature fusion of the growing cranial bone fronts [9].
• Previous studies have shown that TGFbeta signaling regulates the fate of the medial edge epithelium during palatal fusion and postnatal cranial suture closure during skull development [10].

Chemical compound and disease context of Cranial Sutures

• Complications of prostaglandin administration include fever, diarrhoea, hypotension, apnoea, bradycardia, pseudowidening of the cranial sutures, underossification of the calvarial bones, periostitis, and skin edema [1-3] [11].

Biological context of Cranial Sutures

• Relatively little is known about the developmental biology of this process, but genetically determined disorders of premature cranial suture fusion (craniosynostosis) provide one route to the identification of some of the key molecules involved [12].
• Pycnodysostosis (MIM 265800) is a rare, autosomal recessive skeletal dysplasia characterized by short stature, wide cranial sutures, and increased bone density and fragility [13].
• Taken together, these data strongly support the hypothesis that the calvarial DM is regionally differentiated resulting in the up-regulation of osteogenic cytokines and bone ECM molecules in the dural tissues underlying fusing but not patent cranial sutures [14].
• Differential expression patterns of Runx2 isoforms in cranial suture morphogenesis [15].
• To gain insight into the role of these factors in normal growth and development of cranial sutures and the possible etiology of premature cranial suture fusion, we examined the temporal and spatial expression of TGF-beta isoforms during normal cranial suture development in the rat [16].

Anatomical context of Cranial Sutures

• Apert (Ap) syndrome is characterized by premature cranial suture ossification caused by fibroblast growth factor receptor 2 (FGFR-2) mutations [17].
• Sox9 neural crest determinant gene controls patterning and closure of the posterior frontal cranial suture [18].
• The constitutive activation of the fibroblast growth factor (FGF) signaling pathway accelerates osteoblast differentiation and results in premature cranial suture closure [19].
• In summary, the simultaneous expression of FGFR 1-3 genes in cranial sutures might explain their involvement in craniosynostosis syndromes, whereas the specific expression of FGFR 3 in chondrocytes does correlate with the involvement of FGFR 3 mutations in inherited defective growth of human long bones [20].
• FGFR-3 was expressed a lot within the periosteal margins and cranial sutures but not within either the EOMs or the optic nerve sheath [21].

Associations of Cranial Sutures with chemical compounds

• METHODS: Computed tomographic scans demonstrated erosion of the inner table of the cranium and sclerosis of the cranial sutures, particularly the coronal suture [22].
• Error estimation of the fractal dimension measurements of cranial sutures [23].
• METHODS: Serial 3D ultrasound examinations were evaluated for visibility of fetal cranial sutures and fontanels, image quality and possible influencing parameters in the second half of pregnancy [24].
• We therefore investigated whether or not the cranial sutures show accelerated closure and how the local growth factors or cytokines participate and function in local bone metabolism after administration of exogenous excess thyroid hormone in a rat model [25].
• Cranial suture morphology was examined in hematoxylin and eosin-stained sections and in cleared whole-mount specimens, double stained with alizarin red S and Alcian blue [26].

Gene context of Cranial Sutures

• We compare Dlx5 expression pattern with that of several genes involved in mouse cranial suture regulation [27].
• Coincidently, blocking of the Erk pathway also significantly retarded FGF2-accelerated cranial suture closure [28].
• Twist1 dimer selection regulates cranial suture patterning and fusion [29].
• Heterozygous Bey mice are viable and fertile but show facial shortening with increased interorbital distance and precocious closure of several cranial sutures (craniosynostosis) [30].
• Transcript analysis demonstrates that expression of both Fgf3 and Fgf4 is up-regulated in the cranial sutures of Bey mice [30].

Analytical, diagnostic and therapeutic context of Cranial Sutures

• To further analyze regional differentiation of cranial suture-associated dural cells, we established dural cell cultures from fusing and patent rat cranial sutures in N6 rats and evaluated the expression of osteogenic cytokines (TGF-beta1 and fibroblast growth factor 2 [FGF-2]) and collagen I [14].
• This approach may be refined for tissue engineering of cranial sutures for craniosynostosis patients [31].
• The purpose of this experiment was to investigate by immunohistochemistry the protein production of these receptors as well as of their most prevalent ligand, basic fibroblast growth factor (bFGF), before, during, and after sutural fusion in rat cranial sutures [32].
• We demonstrate that Twist heterozygous mice exhibit fusions of the coronal suture and other cranial suture abnormalities, indicating that Twist heterozygous mice constitute a better animal model for Saethre-Chotzen syndrome than was previously appreciated [33].
• RESULTS: Histology of the wild-type cranial sutures (control) showed suture patency (score of 0%) for all coronal and sagittal sutures at 0 days, 7 days, and 14 days of organ culture [34].

References