Fixation methods and immunolabeling for cilia proteins in ciliary and extraciliary locations.

Primary cilia are complex organelles, usually singularly located on cell surfaces that are now known to be important for signaling and whose defect is implicated in a category of developmental diseases known as ciliopathies. They are composed of a microtubule axoneme and contain a cilia membrane that is unique and distinct from the plasma membrane. Primary cilia also have their own transport system termed the intraflagellar transport (IFT) system that allows for proteins to be trafficked along the microtubule axoneme in either an anterograde or retrograde manner.

Primary Cilia Reconsidered in the Context of Ciliopathies: Extraciliary and Ciliary Functions of Cilia Proteins Converge on a Polarity theme?

Once dismissed as vestigial organelles, primary cilia have garnered the interest of scientists, given their importance in development/signaling, and for their implication in a new disease category known as ciliopathies. However, many, if not all, "cilia" proteins also have locations/functions outside of the primary cilium. These extraciliary functions can complicate the interpretation of a particular ciliopathy phenotype: it may be a result of defects at the cilium and/or at extraciliary locations, and it could be broadly related to a unifying cellular process for these proteins, such as polarity.

Primary cilia proteins: ciliary and extraciliary sites and functions.

Primary cilia are immotile organelles known for their roles in development and cell signaling. Defects in primary cilia result in a range of disorders named ciliopathies. Because this organelle can be found singularly on almost all cell types, its importance extends to most organ systems.

Fixation methods can differentially affect ciliary protein immunolabeling.

Background: Primary cilia are immotile, microtubule-based organelles present on most cells. Defects in primary cilia presence/function result in a category of developmental diseases referred to as ciliopathies. As the cilia field progresses, there is a need to consider both the ciliary and extraciliary roles of cilia proteins.