Sensory functions of motile cilia and implication for bronchiectasis

Jain, Raksha; Javidan-Nejad, Cylen; Alexander‐Brett, Jennifer; Horani, Amjad; Cabellon, Michele C.; Walter, Michael; Brody, Steven L.

doi:10.2741/s320

Cited by 34 publications

(15 citation statements)

References 71 publications

(95 reference statements)

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“…The addition of sputum from patients with bronchiectasis to nasal epithelial fragments suspended in tissue culture medium results in reduced ciliary beat frequency, but improves after treatment with antibiotics (132). It has been proposed that defective signaling of sensory proteins in motile cilia may lead to ciliopathy and development of bronchiectasis (133). …”

Section: Acquired Disorders Of Airway Ciliamentioning

confidence: 99%

Cilia Dysfunction in Lung Disease

Tilley

Walters²,

Shaykhiev³

et al. 2015

Annu. Rev. Physiol.

342

308

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A characteristic feature of the human airway epithelium is the presence of ciliated cells bearing motile cilia, specialized cell surface projections containing axonemes comprised of microtubules and dynein arms, which provide ATP-driven motility. In the airways, cilia function in concert with airway mucus to mediate the critical function of mucociliary clearance, cleansing the airways of inhaled particles and pathogens. The prototypical disorder of respiratory cilia is primary ciliary dyskinesia, an inherited disorder that leads to impaired mucociliary clearance, repeated chest infections, and progressive destruction of lung architecture. Numerous acquired lung diseases are also marked by abnormalities in both cilia structure and function. In this review we summarize current knowledge regarding airway ciliated cells and cilia, how they function to maintain a healthy epithelium, and how disorders of cilia structure and function contribute to inherited and acquired lung disease.

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Section: Acquired Disorders Of Airway Ciliamentioning

confidence: 99%

Cilia Dysfunction in Lung Disease

Tilley

Walters²,

Shaykhiev³

et al. 2015

Annu. Rev. Physiol.

342

308

View full text Add to dashboard Cite

show abstract

“…Abnormal ciliary flow in these organs can result in hydrocephalus, infection, and infertility, respectively. Motile cilia can also function as sensory organelles; various receptors, including those for bitter taste and for progesterone, are expressed in motile cilia in respiratory epithelia and fallopian tube, respectively [36–38]. …”

Section: Primary and Motile Ciliamentioning

confidence: 99%

Planar Organization of Multiciliated Ependymal (E1) Cells in the Brain Ventricular Epithelium

Ohata

Alvarez-Buylla

2016

Trends in Neurosciences

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Cerebrospinal fluid (CSF) continuously flows through the cerebral ventricles, a process essential for brain homeostasis. Multiciliated ependymal (E1) cells line the walls of the ventricles and contribute importantly to CSF flow through ciliary beating. Key to this function is E1 cells’ rotational and translational planar cell polarity (PCP). Defects in E1 cells’ PCP can result in abnormal CSF accumulation and hydrocephalus. Here we integrate recent data on the roles of early CSF flow in the embryonic ventricles, PCP regulators (e.g. Vangl2 and Dishevelled), and cytoskeletal networks in the establishment, refinement, and maintenance of E1 cells’ PCP. E1 cells’ planar organization mechanisms could explain how CSF flow contributes to brain function and may help in the diagnosis and prevention of hydrocephalus.

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“…For example, blindness due to mutations in the retinitis pigmentosa GTPase regulator gene ( RPGR ) is associated with motile cilia dysfunction (17). Further overlap in function is suggested by finding the expression of the polycystic kidney disease genes in motile cilia and an association with bronchiectasis in those individuals with cystic kidney disease (18, 19). Motile cilia on human respiratory epithelia possess several members of the family of bitter taste receptors, identical to those in the tongue and nose (20).…”

Section: Cilia Structure and Functionmentioning

confidence: 99%

Picking up speed: advances in the genetics of primary ciliary dyskinesia

2013

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Abnormal ciliary axonemal structure and function are linked to the growing class of genetic disorders collectively known as ciliopathies, and our understanding of the complex genetics and functional phenotypes of these conditions has rapidly expanded. While progress in genetics and biology has uncovered numerous cilia-related syndromes, primary ciliary dyskinesia (PCD) remains the sole genetic disorder of motile cilia dysfunction. The first disease-causing mutation was described just thirteen years ago, and since that time the pace of gene discovery has quickened. These mutations separate into genes that encode axonemal motor proteins, structural and regulatory elements, and cytoplasmic proteins that are involved in assembly and preassembly of ciliary elements. These findings have yielded novel insights into the processes involved in ciliary assembly, structure, and function, which will allow us to better understand the clinical manifestations of primary ciliary dyskinesia. Moreover, advances in techniques for genetic screening and sequencing are improving diagnostic approaches. In this manuscript, we will describe the structure, function, and emerging genetics of respiratory cilia, review the genotype-phenotype relationships of motor ciliopathies, and explore the implications of recent discoveries for diagnostic testing for primary ciliary dyskinesia.

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Sensory functions of motile cilia and implication for bronchiectasis

Cited by 34 publications

References 71 publications

Cilia Dysfunction in Lung Disease

Cilia Dysfunction in Lung Disease

Planar Organization of Multiciliated Ependymal (E1) Cells in the Brain Ventricular Epithelium

Picking up speed: advances in the genetics of primary ciliary dyskinesia

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