| Description |
Cilia are microtubule-based organelles involved in a variety of processes, including sensing and motility. Mutations disrupting ciliary function and structure cause human diseases, such as cancer and a group of syndromes collectively called “ciliopathies”. In those syndromes, which may result from mutations in single or multiple genes, cilia function is defective and single or multiple organs can be affected. A mature centriole converts to basal body to grow the cilium. Very little is known about how the centriole is converted to a basal body and how that conversion allows the formation of diverse cilia, such as motile and immotile structures. This conversion includes the growth of a transition zone, where many human-ciliopathy proteins localize, at the distal end of centriole. The mechanisms of transition zone assembly, the extent of their variability, and their mechanisms of diversification in different cilia types are mostly unknown. Drosophila displays both motile and immotile ciliated cells, allowing us to understand the mechanisms behind the generation of diverse ciliary structures. We describe the diversity and conservation of structural and molecular components of the basal bodies in different ciliated cells in Drosophila and show how some disease associated molecules, such as CEP290, CEP164 and BLD10 are differentially involved in their assembly and maintenance. Our findings provide new hypotheses for the genesis of organ/tissue specific defects in several human ciliopathies.
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