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Publication by Bestcilia partner: defects of mucociliary clearance not only caused by problems in structure of cilia

Bestcilia partner from Münster University in Germany has authored a publication on CCNO, a novel gene related to the process of mucociliary airway clearance. In contrast to other genes identified so far by the team of Prof. Heymut Omran, mutations in CCNO gene do not cause changes in the structure of motile cilia, but affect the process of motile cilia generation.

Using a whole-exome sequencing strategy, the group of Prof. Heymut Omran  analysed 16 cases of chronic lung diseases affecting airway clearance. The recessive CCNO mutations discovered in this group of patients have caused a significant reduction in the number of multiple motile cilia on the surface of respiratory epithelial cells. Careful subcellular analyses have clarified that CCNO  mutations affect the generation and placement of mother centriole epithelial cells, which is the first step in the generation of motile cilia. Research on CCNO is reportedly the first to indicate that hereditary lung diseases can also be caused by reduced number of multiple motile cilia.

Full article can be accessed here