The scope of CILIAGATE is to understand the molecular mechanism that allows the transition zone (TZ), a conserved protein complex with unknown molecular structure, to maintain a specific lipid and protein composition in cilia organelles.
Cilia enable motility and sensory perception and are dysfunctional in rare human diseases known as ciliopathies that affects multiple organs leading to blindness, deafness, obesity, mental retardation, and renal dysfunctions.
Most ciliopathies maps to TZ that lies at the base of cilia and allows selective diffusion and transport of proteins and lipids between cytosol and cilia by an unknown gating mechanism.
Here I have predicted the structure of the TZ complex from the extracellular space to cilium’s core and revealed that consists of more than 288 proteins organized in modules.
CILIAGATE aims to provide experimental validation for the predicted structural model of TZ and dissect its gating mechanism in cellular models such as neurons and RPE1 cells.