Recombinant mouse periostin ameliorates coronal sutures fusion in Twist1^+/- mice.

ABSTRACT:

Background

Saethre-Chotzen syndrome is an autosomal dominantly inherited disorder caused by mutations in the twist family basic helix-loop-helix transcription factor 1 (TWIST1) gene. Surgical procedures are frequently required to reduce morphological and functional defects in patients with Saethre-Chotzen syndrome. Therefore, the development of noninvasive procedures to treat Saethre-Chotzen syndrome is critical. We identified that periostin, which is an extracellular matrix protein that plays an important role in both bone and connective tissues, is downregulated in craniosynostosis patients.

Methods

We aimed to verify the effects of different concentrations (0, 50, 100, and 200 μg/l) of recombinant mouse periostin in Twist1^+/- mice (a mouse model of Saethre-Chot

SUBMITTER: Bai S 

PROVIDER: S-EPMC5905175 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature