Erwin Pauws
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View article: Unravelling the difference in craniofacial morphology of Yucatan miniature and standard pigs during postnatal ontogeny
Unravelling the difference in craniofacial morphology of Yucatan miniature and standard pigs during postnatal ontogeny Open
The Yucatan miniature pig has become a preferred model for craniofacial research due to its anatomical and physiological similarities to humans. However, the factors driving midfacial hypoplasia in Yucatans during postnatal ontogeny remain…
View article: Chiari II brain malformation is secondary to open spina bifida
Chiari II brain malformation is secondary to open spina bifida Open
Chiari II brain malformation affects 90% of children with open spina bifida. The hindbrain herniates into the vertebral canal leading to hydrocephalus and occasional respiratory emergency. Chiari II is not confined to the hindbrain, but is…
View article: Cranial bone microarchitecture in a mouse model for syndromic craniosynostosis
Cranial bone microarchitecture in a mouse model for syndromic craniosynostosis Open
Crouzon syndrome is a congenital craniofacial disorder caused by mutations in the Fibroblast Growth Factor Receptor 2 (FGFR2). It is characterized by the premature fusion of cranial sutures, leading to a brachycephalic head shape, and midf…
View article: <scp>BounTI</scp> (boundary‐preserving threshold iteration): A user‐friendly tool for automatic hard tissue segmentation
<span>BounTI</span> (boundary‐preserving threshold iteration): A user‐friendly tool for automatic hard tissue segmentation Open
X‐ray Computed Tomography (CT) images are widely used in various fields of natural, physical, and biological sciences. 3D reconstruction of the images involves segmentation of the structures of interest. Manual segmentation has been widely…
View article: Multiscale mechanical characterisation of the craniofacial system under external forces
Multiscale mechanical characterisation of the craniofacial system under external forces Open
Premature fusion of craniofacial joints, i.e. sutures, is a major clinical condition. This condition affects children and often requires numerous invasive surgeries to correct. Minimally invasive external loading of the skull has shown som…
View article: Synchondrosis fusion contributes to the progression of postnatal craniofacial dysmorphology in syndromic craniosynostosis
Synchondrosis fusion contributes to the progression of postnatal craniofacial dysmorphology in syndromic craniosynostosis Open
Syndromic craniosynostosis (CS) patients exhibit early, bony fusion of calvarial sutures and cranial synchondroses, resulting in craniofacial dysmorphology. In this study, we chronologically evaluated skull morphology change after abnormal…
View article: TBX22 missense mutations found in patients with x-linked cleft palate affect DNA binding, sumoylation, and transcriptional repression
TBX22 missense mutations found in patients with x-linked cleft palate affect DNA binding, sumoylation, and transcriptional repression Open
The T-box transcription factor TBX22 is essential for normal craniofacial development, as demonstrated by the finding of nonsense, frameshift, splice-site, or missense mutations in patients with X-linked cleft palate (CPX) and ankyloglossi…
View article: Mechanical loading of cranial joints minimizes the craniofacial phenotype in Crouzon syndrome
Mechanical loading of cranial joints minimizes the craniofacial phenotype in Crouzon syndrome Open
Children with syndromic forms of craniosynostosis undergo a plethora of surgical interventions to resolve the clinical features caused by the premature fusion of cranial sutures. While surgical correction is reliable, the need for repeated…
View article: Overexpression of <i>Fgfr2c</i> causes craniofacial bone hypoplasia and ameliorates craniosynostosis in the Crouzon mouse
Overexpression of <i>Fgfr2c</i> causes craniofacial bone hypoplasia and ameliorates craniosynostosis in the Crouzon mouse Open
FGFR2c regulates many aspects of craniofacial and skeletal development. Mutations in the FGFR2 gene are causative of multiple forms of syndromic craniosynostosis, including Crouzon syndrome. Paradoxically, mouse studies have shown that the…
View article: Mouse Models of Syndromic Craniosynostosis
Mouse Models of Syndromic Craniosynostosis Open
Craniosynostosis is a common craniofacial birth defect. This review focusses on the advances that have been achieved through studying the pathogenesis of craniosynostosis using mouse models. Classic methods of gene targeting which generate…
View article: Tournaments
Tournaments Open
Homozygous LCDmut develop neurodegenerative phenotypes including a) decreased grip strength and b) muscle force, together with c) reduced number of surviving motor units and d) positive p62 inclusions in the spinal cord Conclusion: Mutatio…
View article: Predicting calvarial growth in normal and craniosynostotic mice using a computational approach
Predicting calvarial growth in normal and craniosynostotic mice using a computational approach Open
During postnatal calvarial growth the brain grows gradually and the overlying bones and sutures accommodate that growth until the later juvenile stages. The whole process is coordinated through a complex series of biological, chemical and …
View article: Analysis of the Fgfr2C342Y mouse model shows condensation defects due to misregulation of Sox9 expression in prechondrocytic mesenchyme
Analysis of the Fgfr2C342Y mouse model shows condensation defects due to misregulation of Sox9 expression in prechondrocytic mesenchyme Open
Syndromic craniosynostosis caused by mutations in FGFR2 is characterised by developmental pathology in both endochondral and membranous skeletogenesis. Detailed phenotypic characterisation of features in the membranous calvarium, the endoc…