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Why Scientists Are Blaming Cilia for Human Disease

At first blush, cilia seem relatively innocuous. As they beat back and forth outside the cell, coordinated brushes of so-called motile cilia regulate fluid flow nearby. But almost all human cells also have one primary, or nonmotile, cilium that functions more like a molecular antenna. The primary cilium is an internally dynamic structure, packed with proteins that detect and convey important messages to its cell about the local environment. “The signaling machinery is concentrated in the cilia,” Witman says. “All in this very tightly controlled, constrained space.”

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 Making sense of cilia in disease: The human ciliopathies

Ubiquitous in nature, cilia and flagella comprise nearly identical structures with similar functions. The most obvious example of the latter is motility: driving movement of the organism or particle flow across the epithelial surface in fixed structures. In vertebrates, such motile cilia are evident in the respiratory epithelia, ependyma, and oviducts. For over a century, non-motile cilia have been observed on the surface of most vertebrate cells but until recently their function has eluded us.

Articles of Interest

Cilia in the brain may be busier than previously thought

Nerve cells in the brain make elaborate connections and exchange lightning-quick messages that captivate scientists. But these cells also sport simpler, hairlike protrusions called cilia. Long overlooked, the little stubs may actually have big jobs in the brain.

Cilia Structure and Function in Human Disease

Ciliary dysfunction causes a large group of developmental and degenerative human diseases known as ciliopathies. These diseases reflect the critical roles that cilia play in sensing the environment and in force generation for motility. Sensory functions include our senses of vision and olfaction. In addition, primary and motile cilia throughout our body monitor the environment allowing cells to coordinate their biology with the cells around them.

Cystic Fibrosis and Primary Ciliary Dyskinesia Share Extensive Similarities: Is It Time to Place Them Under a Common Umbrella?

Both conditions impair different hydrodynamic components of mucociliary clearance, predispose to recurrent respiratory infections, and often lead to the development of bronchiectasis.

Seriously Cilia: A Tiny Organelle Illuminates Evolution, Disease and Intercellular Communication

Reflecting their diverse motility and signaling functions, compromised cilia cause a diverse range of diseases collectively called ciliopathies.

The Primary Cilium: An Orphan Organelle Finds a Home

For a long time, almost all attention was on the motile cilia because their function was readily observable. But scientists, starting with Alexander Kowalevsky had reported the presence of single (nonmotile) cilia in a variety of vertebrate cells (Kowalevsky 1867)

The roles of cilia in developmental disorders and disease

Ciliary function has recently been shown to be crucial in an increasingly diverse range of biological functions. The location of the cilium on the cell surface effectively positions it to function as an environmental sensor and vehicle to transfer information that affects important developmental decisions.

Resources

Ciliopathy Alliance

The Ciliopathy Alliance brings together patient support groups, researchers, doctors and allied health professionals representing patients and families living with and affected by ciliopathies - diseases caused by defects in the function or structure of cilia.