• Home
  • Search Results
  • The ciliary protein Ftm is required for ventricular wall and septal development.

The ciliary protein Ftm is required for ventricular wall and septal development.

PloS one (2013-03-08)
Christoph Gerhardt, Johanna M Lier, Stefanie Kuschel, Ulrich Rüther

Ventricular septal defects (VSDs) are the most common congenital heart defects in humans. Despite several studies of the molecular mechanisms involved in ventricular septum (VS) development, very little is known about VS-forming signaling. We observed perimembranous and muscular VSDs in Fantom (Ftm)-negative mice. Since Ftm is a ciliary protein, we investigated presence and function of cilia in murine hearts. Primary cilia could be detected at distinct positions in atria and ventricles at embryonic days (E) 10.5-12.5. The loss of Ftm leads to shortened cilia and a reduced proliferation in distinct atrial and ventricular ciliary regions at E11.5. Consequently, wall thickness is diminished in these areas. We suggest that ventricular proliferation is regulated by cilia-mediated Sonic hedgehog (Shh) and platelet-derived growth factor receptor α (Pdgfrα) signaling. Accordingly, we propose that primary cilia govern the cardiac proliferation which is essential for proper atrial and ventricular wall development and hence for the fully outgrowth of the VS. Thus, our study suggests ciliopathy as a cause of VSDs.

Product Number
Product Description

Anti-Actin antibody produced in rabbit, affinity isolated antibody, buffered aqueous solution
Monoclonal Anti-Tubulin, Acetylated antibody produced in mouse, clone 6-11B-1, ascites fluid
Anti-GAPDH antibody, Mouse monoclonal, clone GAPDH-71.1, purified from hybridoma cell culture
Monoclonal Anti-γ-Tubulin antibody produced in mouse, clone GTU-88, ascites fluid
Expand Reverse Transcriptase, solution, recombinant, expressed in E. coli (AP401 (k)), sufficient for 20 reactions, sufficient for 100 reactions
ApopTag Plus Peroxidase In Situ Apoptosis Kit, The ApopTag Plus Peroxidase In Situ Apoptosis Detection Kit detects apoptotic cells by labeling & detecting DNA strand breaks by the indirect TUNEL method.
Anti-Tubulin Antibody, Detyrosinated, Chemicon®, from rabbit