Notum attenuates Wnt/β-catenin signaling to promote tracheal cartilage patterning.

ABSTRACT: Tracheobronchomalacia (TBM) is a common congenital disorder in which the cartilaginous rings of the trachea are weakened or missing. Despite the high prevalence and clinical issues associated with TBM, the etiology is largely unknown. Our previous studies demonstrated that Wntless (Wls) and its associated Wnt pathways are critical for patterning of the upper airways. Deletion of Wls in respiratory endoderm caused TBM and ectopic trachealis muscle. To understand mechanisms by which Wls mediates tracheal patterning, we performed RNA sequencing in prechondrogenic tracheal tissue of Wls^f/f;Shh^Cre/wt embryos. Chondrogenic Bmp4, and Sox9 were decreased, while expression of myogenic genes was increased. We identified Notum, a deacylase that inactivates Wnt ligands, as a targ

SUBMITTER: Gerhardt B 

PROVIDER: S-EPMC5889736 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature