Cystic kidney diseases: all roads lead to the cilium.

Cystic kidney disorders are one of the leading causes of end-stage renal disease. Numerous experimental animal models have been used to understand the disease pathogenesis. Recent advancements in this field have provided a surprising finding: that many of the proteins associated with cystic kidney disease localize to a nearly forgotten organelle, the primary cilium.

A novel dynein light intermediate chain colocalizes with the retrograde motor for intraflagellar transport at sites of axoneme assembly in chlamydomonas and Mammalian cells.

The assembly of cilia and flagella depends on bidirectional intraflagellar transport (IFT). Anterograde IFT is driven by kinesin II, whereas retrograde IFT requires cytoplasmic dynein 1b (cDHC1b). Little is known about how cDHC1b interacts with its cargoes or how it is regulated.

Identification of CHE-13, a novel intraflagellar transport protein required for cilia formation.

Cilia are present on cells of many eukaryotic organisms and recent data in the mouse suggest that ciliary defects can cause severe developmental abnormalities and disease. Studies across eukaryotic systems indicate that cilia are constructed and maintained through a highly conserved process termed intraflagellar transport (IFT), for which many of the proteins involved have yet to be identified. IFT describes the movement of large protein particles consisting of an A and a B complex along the cilia axoneme in anterograde and retrograde directions.