Data from: A liquid-like organelle at the root of motile ciliopathy

Motile ciliopathies are characterized by specific defects in cilia beating
that result in chronic airway disease, subfertility, ectopic pregnancy,
and hydrocephalus. While many patients harbor mutations in the dynein
motors that drive cilia beating, the disease also results from mutations
in so-called Dynein Axonemal Assembly Factors (DNAAFs) that act in the
cytoplasm. The mechanisms of DNAAF action remain poorly defined. Here, we
show that DNAAFs concentrate together with axonemal dyneins and chaperones
into organelles that form specifically in multiciliated cells, which we
term DynAPs, for Dynein Axonemal Particles. These organelles display
hallmarks of biomolecular condensates, and remarkably, DynAPs are enriched
for the stress granule protein G3bp1, but not for other stress granule
proteins or P-body proteins. Finally, we show that both the formation and
the liquid-like behaviors of DynAPs are disrupted in a model of motile
ciliopathy. These findings provide a unifying cell biological framework
for a poorly understood class of human disease genes and add motile
ciliopathy to the growing roster of human diseases associated with
disrupted biological phase separation.