Lim mineralization protein is involved in the premature calvarial ossification in sporadic craniosynostoses.

摘要: Abstract Sporadic mono-sutural craniosynostosis represents a highly prevalent regional bone disorder, where single cranial suture undergoes premature ossification due to generally unclear etiopathogenesis. The LIM mineralization protein (LMP) was recently described as an efficient osteogenic molecule involved in osteoblast differentiation, expressed calvarial tissues upon corticosteroid-osteogenic induction and used potent inducer of formation several animal models. In this study, cells isolated from both prematurely fused physiologically patent sutures children with sporadic craniosynostosis, were vitro paradigmatic model for the study molecular events osteogenesis, focusing on possible role LMP-related signaling. Calvarial mesenchymal-like immunophenotype. Cells displayed increased potential, being able undergo spontaneous response induction, leading nodule formation. expression LMP its target genes (bone morphogenetic protein-2, osteocalcin Runt-related transcription factor 2) significantly up-regulated derived sutures. Upon silencing suture-derived cells, potential along osteo-specific factors decreased, restoring “physiologic” cell behavior. These results suggested that: 1. mesenchymal residing display constitutionally active disposition ossification; 2. basis overactive process may at least part involve deregulation pathway cells.