craniosynostoses/phosphatase

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The effects of tissue-non-specific alkaline phosphatase gene therapy on craniosynostosis and craniofacial morphology in the FGFR2C342Y/+ mouse model of Crouzon craniosynostosis.

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OBJECTIVE Craniosynostosis, the premature fusion of cranial bones, has traditionally been described as a disease of increased bone mineralization. However, multiple mouse models of craniosynostosis display craniosynostosis simultaneously with diminished cranial bone volume and/or density. We propose

Viral Delivery of Tissue Nonspecific Alkaline Phosphatase Diminishes Craniosynostosis in One of Two FGFR2C342Y/+ Mouse Models of Crouzon Syndrome

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Craniosynostosis is the premature fusion of cranial bones. The goal of this study was to determine if delivery of recombinant tissue nonspecific alkaline phosphatase (TNAP) could prevent or diminish the severity of craniosynostosis in a C57BL/6 FGFR2C342Y/+ model of neonatal onset craniosynostosis

Enzyme replacement therapy for congenital hypophosphatasia allows for surgical treatment of related complex craniosynostosis: a case series.

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Hypophosphatasia (HPP) is a rare inherited disorder of bone metabolism that results in the loss of function of the gene coding for tissue-nonspecific alkaline phosphatase (TNSALP). Patients with HPP have defective bone mineralization as well as craniosynostosis that can be seen in the infantile and

Dura in the pathogenesis of syndromic craniosynostosis: fibroblast growth factor receptor 2 mutations in dural cells promote osteogenic proliferation and differentiation of osteoblasts.

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Mutations in fibroblast growth factor receptor 2 (FGFR2), a transmembrane receptor expressed in suture mesenchyme, osteogenic fronts, and dura, have been implicated in the etiopathogenesis of craniosynostosis syndromes. The C278F- and P253R-FGFR2 mutations result in Crouzon and Apert syndromes,

Severe hypophosphatasia due to mutations in the tissue-nonspecific alkaline phosphatase (TNSALP) gene.

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Hypophosphatasia is a rare autosomal recessive inborn error of metabolism characterized by a defective bone mineralisation and deficiency of serum and tissue liver/bone/kidney alkaline phosphatase activity. We report the characterisation of tissue-nonspecific alkaline phosphatase (TNSALP) gene

Force-induced craniosynostosis via paracrine signaling in the murine sagittal suture.

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BACKGROUND The role of genetic phenomena has been given central importance in the development of craniosynostosis. Proponents have dismissed the role of force as a key etiologic factor. Nonetheless, compressive forces on the developing calvarium have been shown to result in premature suture fusion.

Regression modeling to inform cell incorporation into therapies for craniosynostosis.

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Designing an appropriate tissue engineering solution for craniosynostosis (CS) necessitates determination of whether CS-derived cells differ from normal (wild-type, WT) cells and what assays are appropriate to test for differences. Traditional methodologies to statistically compare cellular behavior

Unilateral coronal synostosis: a histomorphometric study.

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OBJECTIVE This histomorphometric study compared the open and prematurely fused side of the coronal suture in subjects with unilateral coronal synostosis (UCS). METHODS Sutures and parasutural bone were obtained from seven subjects with nonsyndromic UCS during operative correction at 3 to 24 months

Craniosynostosis-associated Fgfr2(C342Y) mutant bone marrow stromal cells exhibit cell autonomous abnormalities in osteoblast differentiation and bone formation.

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We recently reported that cranial bones of Fgfr2(C342Y/+) craniosynostotic mice are diminished in density when compared to those of wild type mice, and that cranial bone cells isolated from the mutant mice exhibit inhibited late stage osteoblast differentiation. To provide further support for the

Histopathological and biochemical changes in the sutural region in craniosynostosis.

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OBJECTIVE Histopathological observations and biochemical analysis of sutural bones in nine patients with craniosynostosis were compared with control subjects of the same age range. METHODS Microscopic examination in craniosynostosis showed formation of an active osseous front, with higher

Potential role of PC-1 expression and pyrophosphate elaboration in the molecular etiology of the FGFR-associated craniosynostosis syndromes.

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BACKGROUND Fibroblast growth factor/fibroblast growth factor receptor (FGF/FGFR) signaling is associated with the aberrant mineralization phenotype of the craniosynostosis syndromes. One critical aspect of mineralization involves the elaboration and transport of pyrophosphate into the extracellular

Bone mineralization-dependent craniosynostosis and craniofacial shape abnormalities in the mouse model of infantile hypophosphatasia.

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BACKGROUND Inactivating mutations in tissue-nonspecific alkaline phosphatase (TNAP) cause hypophosphatasia (HPP), which is commonly characterized by decreased bone mineralization. Infants and mice with HPP can also develop craniosynostosis and craniofacial shape abnormalities, although the mechanism

A de novo balanced translocation t(7;12)(p21.2;p12.3) in a patient with Saethre-Chotzen-like phenotype downregulates TWIST and an osteoclastic protein-tyrosine phosphatase, PTP-oc.

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Saethre-Chotzen syndrome (SCS) is an autosomal dominant craniosynostosis syndrome with variable expression. Here we report on a female infant with a de novo balanced translocation 46, XX, t(7;12)(p21.2;p12.3) and presenting at birth brachycephaly, antimongolic palpebral fissures, ocular

Enzyme replacement for craniofacial skeletal defects and craniosynostosis in murine hypophosphatasia.

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Hypophosphatasia (HPP) is an inborn-error-of-metabolism disorder characterized by deficient bone and tooth mineralization due to loss-of function mutations in the gene (Alpl) encoding tissue-nonspecific alkaline phosphatase (TNAP). Alpl(-/-) mice exhibit many characteristics seen in infantile HPP

Improvement of the skeletal and dental hypophosphatasia phenotype in Alpl-/- mice by administration of soluble (non-targeted) chimeric alkaline phosphatase.

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Hypophosphatasia (HPP) results from ALPL gene mutations, which lead to a deficiency of tissue-nonspecific alkaline phosphatase (TNAP), and accumulation of inorganic pyrophosphate, a potent inhibitor of mineralization that is also a natural substrate of TNAP, in the extracellular space. HPP causes

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