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Cilia

Chapter · July 2019

DOI: 10.1007/978-3-319-47829-6_1421-1

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All India Institute of Medical Sciences Jodhpur
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C
Cilia
Hare Krishna
Department of Anatomy, All India Institute of
Medical Sciences, Jodhpur, Rajasthan, India
Synonyms
Fibril; Filament; Footlet; String; Tendril; Thread
Definition
Cilia are membrane-bounded, centriole-derived,
microtubule-containing, hair-like extensions of
the apical plasma membrane.
Introduction
Cilia are common surface modifications present
on nearly all cell types in mammals. The term
cilium (Latin word, meaning “eyelash”) was first
used by Otto Friedrich Muller in 1786. Cilia were
first observed by Antoni van Leeuwenhoek in
1674–1675; he called them animalcules which
mean motile thin little feet or little legs
(Sengupta 2017).
Initially, the cilia would not be able to gain the
importance that it deserved and was relegated to a
very minor role in moving out the mucous from
respiratory tract. Over the years of research, they
© Springer Nature Switzerland AG 2019
J. Vonk, T. K. Shackelford (eds.), Encyclopedia of Animal Cognition and Behavior,
https://doi.org/10.1007/978-3-319-47829-6_1421-1
found that the cilia were present virtually in every
tissue including fallopian tubes, neurons,
chondrocytes, limb bud ectoderm and mesenchy-
mal cells, ependymal cells of brain ventricles,
kidney tubule epithelial cells, pancreatic duct
and islet cells, liver cells, and even many tissue
culture cells. In majority of these organs, these
cilia were nonmotile so they were considered as
nonfunctional (Marshall and Nonaka 2006).
But recently, cilia have important role in mig-
ration of cells during development of embryo,
sensory signaling pathways, and its association
with number of disease syndromes; these organ-
elles become center of attraction for biologists
(Bloodgood 2009).
Classification
Cilia are classified into three categories based on
their functional characteristics:
1. Motile cilia
2. Primary cilia
3. Nodal cilia
Motile cilia
Motile cilia are most extensively studied of all the
types and are usually found on the apical domains
of epithelial cells of the trachea, ependymal
cells of brain ventricles, and on cells lining the
fallopian tube and epididymis (Davenport and
Yoder 2005). Cilia work in more precise and
2 Cilia
organized fashion and are responsible for fluid
and cell movement in the body at various sites
such as clearance of mucus in respiratory tract,
cerebrospinal fluid movement in the ventricles of
brain, and the transport of ovum and sperm in their
corresponding reproductive tracts. Motile cilia
have typical 9 + 2 axonemal organization where
nine microtubule doublets surround a central pair
of microtubules. This central pair has motor pro-
tein which is important for the generation of forces
inducing motility (Porter and Sale 2000).
Function of Motile Cilia
Cilia are motile structure which aids in transport
of cells, proteins, foreign particles, and secretions
from the epithelial surfaces. They are responsible
for the forward movement of the sperm.
Primary Cilia (Monocilia)
Most of the vertebrate cells possess a single non-
motile cilium per cell called the primary cilium.
This microtubule-filled organelle project several
microns from the cell surface (Pan and Snell
2007). They are also found in some epithelial
cells such as the epithelial cells of the rete testis
in the male reproductive tract, epithelial cells in
the biliary tract, epithelial cells of kidney tubules,
ependymal cells lining the ventricles of the central
nervous system, the connecting stalk of photo-
receptor cells in the retina, and the vestibular
hair cells of the ear. They contain 9 + 0 pattern
of microtubule instead of 9 + 2 arrangement.
These are nonmotile because they lack central
pair of microtubule and the microtubule-
associated motor proteins (Pawlina 2016).
Function of Primary Cilia
Primary cilia act as antennae that receive signals
from the surroundings. The receptors present on
primary cilium mediate light sensations, odorant,
and sound perception in multiple organs in the
body. The primary cilia also contain various
cilia-specific receptors, ion channels, and signal-
ing molecules which act as chemosensors,
mechanosensors, and osmosensors. This remote
information is converted into signaling cascades
that are initiated within the ciliary compartment
and then send to the cell cytoplasm.
They also play an important role in the cell
cycle and cell differentiation and for normal tissue
morphogenesis in developing tissues (Satir and
Christensen 2007).
Nodal Cilia
Nodal cilia are found on the bilaminar embryonic
disc at the time of gastrulation around the primi-
tive node in the developing embryo. The axone-
mal internal architecture (9+0 axonemes) of nodal
cilium is similar to that of the primary cilium; the
only difference is the ability of nodal cilium to
perform rotational movement due to presence
of motor proteins dyneins or kinesins. (Supp
et al. 1997). They generate a leftward flow across
the node which is detected by sensory receptors
on the left side of the body. After that they initiate
signaling mechanisms that differ from those on
the right side of the embryo. Any defect in this
mechanism causes the left–right asymmetry of
internal organs (Nonaka et al. 2002).
Function of Nodal Cilia
They are essential in developing left-right
asymmetry of internal organs in the developing
embryo.
Structure of Cilia
Ultrastructurally, Cilium is composed of internal
core of microtubules, called axoneme on longitu-
dinal section. But on cross-sectional view, it con-
tains either nine pairs of microtubules or doublets
of nine microtubules surrounding two central
microtubules. The microtubules composing
each doublet are composed of two types of
microtubule – A microtubule and B microtubule.
The A microtubule is composed of 13 tubulin
protofilaments whereas the B microtubule is
composed of 10 tubulin protofilaments (Fig. 1).
Each microtubule A exhibits a pair of “arms”
that contain a motor protein “dynein.” The protein
“nexin” permanently links the microtubule A with
the microtubule B of adjacent doublets. Each of
the nine doublets give rise to radial spokes, toward
the two central microtubules. The 9 +2 microtu-
bule arrangement is continuous from the tip of the
Cilia
Cilia, Fig. 1 Structure of Central bridge
motile cilia. (Source:
https://commons. Central singlet
microtubules
wikimedia.org/w/index.
php?curid=3145956)
Triplet centriole
cilium to its base, whereas the outer paired micro-
tubules join the basal body. Basal body with the
help of basal body–associated structures anchors
cilia in the apical cell cytoplasm.
Ciliary Diseases
1. Primary ciliary dyskinesia or immotile cilia
syndrome: Immotile cilia affect various system
of the body.
(a) Respiratory system: chronic respiratory
distress with persistent cough, bronchitis,
asthma, and sinusitis
(b) Ear: Otitis media
(c) CNS: Hydrocephalus internus or transient
dilatation of inner brain ventricles
(d) Reproductive system: sterility
2. Polycystic kidney disease: In this disease, there
is mutation in genes which are required for
polycystin1, polycystin 2, and fibrocystin pro-
tein synthesis. Polycystin-1, located within the
cilium (9+0 arrangement), acts as a mechano or
chemo sensor and help in transmission of
the signal from the extracellular fluid comp-
artment to the interacting Ca2+-channel
polycystin-2. Polycystin-2 then mediates suffi-
cient Ca2+ influx to activate intracellular
ryanodine receptors resulting in
3
Plasma
membrane
Outer dynein
Inner dynein
Nexin
Spoke head
Radial Spoke
Subfiber B
Subfiber A
Basal body
intracellular Ca2+ release. Mutations in Poly-
cystin-1 or Polycystin-2 might disable cilia-
mediated mechanosensation, which is normally
required for tissue morphogenesis. Thus, these
mutations in Polycystin-1 or Polycystin-2 can
cause polycystic kidney disease (Sun
et al. 2004).
3. Kartagener’s syndrome: There is absence
of dynein arms which result in failure of
mucociliary transport system.
4. Situs inversus: due to defect in nodal cilia.
5. Other emerging ciliary diseases due to ciliary
involvement:
(A) Retinitis pigmentosa
(B) Bardet–Biedl syndrome (Beales 2005)
Conclusion
Cilia are emerging as omnipresent organelles
which act as antennae sensing the external envi-
ronment. Their defects in structure are responsible
for an increasing number of disease syndromes.
Almost all mammalian cells have a cilium; the
functions of cilia in many organs are now known
but in many other organs have not been known at
all. We are only just beginning to understand, so a
lot of researches are also needed to know other
facts about cilia.
4 Cilia
References
Beales, P. L. (2005). Lifting the lid on Pandora’s box: the
Bardet-Biedl syndrome. Current Opinion in Genetics
& Development, 15(3), 315–323.
Bloodgood, R. A. (2009). From central to rudimentary to
primary: the history of an underappreciated organelle
whose time has come. The primary cilium. Methods in
Cell Biology, 94, 2–52. Academic Press.
Davenport, J. R., & Yoder, B. K. (2005). An incredible
decade for the primary cilium: A look at a once-
forgotten organelle. American Journal of Physiology -
Renal Physiology, 289(6), 1159–1169.
Marshall, W. F., & Nonaka, S. (2006). Cilia: tuning in to
the cell’s antenna. Current Biology, 16(15), 604–614.
Nonaka, S., Shiratori, H., Saijoh, Y., & Hamada, H. (2002).
Determination of left right patterning of the mouse
embryo by artificial nodal flow. Nature, 418(6893), 96.
Pan, J., & Snell, W. (2007). The primary cilium: Keeper of
the key to cell division. Cell, 129(7), 1255–1257.
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Pawlina, W. (2016). Histology a text and atlas (7th ed.,
pp. 111–118). Philadelphia: Wolters Kluwer.
Porter, M. E., & Sale, W. S. (2000). The 9+ 2 axoneme
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Satir, P., & Christensen, S. T. (2007). Overview of structure
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Sun, Z., Amsterdam, A., Pazour, G. J., Cole, D. G.,
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