The cranial base in craniofacial development: a gene therapy study.
J Dent Res
; 86(10): 956-61, 2007 Oct.
Article
en En
| MEDLINE
| ID: mdl-17890671
The etiology of midface retrusion remains largely unclear. We hypothesized that the cranial base synchondroses play a key role in the development of the craniofacial skeleton in the Sandhoff mouse model. We observed that developmental abnormalities of the cranial base synchondroses involving proliferative chondrocytes are important in craniofacial growth and development. Neonatal restitution of beta-hexosaminidase in mutant mice by gene therapy successfully ameliorated the attendant skeletal defects and restored craniofacial morphology in vivo, suggesting this as a critical temporal window in craniofacial development. Analysis of our data implicates parathyroid-related peptide (PTHrP) and cyclo-oxygenase-2 (COX-2) as possible factors underlying the development of the aforementioned skeletal defects. Hence, timely restitution of a genetic deficiency or, alternatively, the restoration of PTHrP or cyclo-oxygenase activity by the administration of PTH and/or non-steroidal anti-inflammatory drugs or COX-2 selective inhibitors to affected individuals may prove beneficial in the management of midface retrusion.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Enfermedad de Sandhoff
/
Beta-N-Acetilhexosaminidasas
/
Base del Cráneo
/
Huesos Faciales
/
Desarrollo Maxilofacial
Tipo de estudio:
Prognostic_studies
Límite:
Animals
Idioma:
En
Revista:
J Dent Res
Año:
2007
Tipo del documento:
Article
País de afiliación:
Estados Unidos
Pais de publicación:
Estados Unidos