In eukaryotes, cilia and flagella are highly conserved microtubule-based organelles that
play essential roles in cell and fluid motility, as well as in cell signaling. An increasing
number of inherited disorders in humans have been linked to defects in cilia assembly or function, collectively referred to as ciliopathies. These disorders are remarkable for their wide spectrum of clinical manifestations, ranging from chronic respiratory infections to polycystic kidney disease, obesity, diabetes, retinal degeneration, and intellectual disability.
A distinct subset of the most severe ciliopathies arises from defects at the ciliary base, which is composed of the basal body, transition fibers, and the transition zone (TZ). The assembly of these structures is highly conserved throughout evolution. While many proteins involved in the assembly and function of the ciliary base have been identified, their complex interactions remain poorly understood