1062-3604/05/3603- © 2005 Pleiades Publishing, Inc.
Russian Journal of Developmental Biology, Vol. 36, No. 3, 2005, pp. 152–160. Translated from Ontogenez, Vol. 36, No. 3, 2005, pp. 190–198.
Original Russian Text Copyright © 2005 by Domaratskii, Uvakina, Volkov, Onishchenko.
Numerous centrioles are formed during differentia-
tion of the ciliary epithelial cells. Two main pathways
of increase in the number of such structures have been
described (Vorobjev and Nadezhdina, 1987). The ﬁrst
(acentriolar) involves the formation of centrioles
with the help of amorphous electron-dense
structures of the deuterosome type, which may be pre-
cursors for the formation of centrioles (Sorokin, 1968;
Steinman, 1968; Anderson and Brenner, 1971; Youson,
1982; Loots and Nel, 1989; Dirksen, 1991; Domar-
atskii and Onishchenko, 2002). The second pathway is
centriolar. In this case, the already present centrioles
serve as a source of the formation of new centrioles.
This pathway was described in the epithelium of the
oviduct of rhesus macaque embryos (Anderson and
Brenner, 1971), where it is represented as a minor path-
way of the formation of basal bodies, in addition to the
more productive pathway: formation of centrioles
accompanied by the mass production of basal
Note that ciliogenesis in mammalian cells has been
described for embryonic tissues. A question arises
whether ciliogenesis is possible in postnatal develop-
ment of mammals, similar to that described in molluscs
(Domaratskii and Onishchenko, 2002). Ciliogenesis in
the ciliary epithelium has not yet been studied in post-
natal development of mammals. In the culture of
human respiratory epithelium, the formation of cilia
was described, including all stages of centriole forma-
tion and ciliogenesis characteristic for the formation of
cilia in embryogenesis (Jorissen
, 1990). However,
it remains unclear whether this process takes place
. We studied the biopsied materials of bronchus
epithelium in children with ciliary dyskinesia syn-
drome. The main attention was paid to Kartagener syn-
drome, a variant of ciliary dyskinesia.
Kartagener syndrome, a rare genetic disease with
autosome-recessive type of inheritance (Gorham and
Merselis, 1959) is considered as a variant of the more
widespread phenomenon, primary ciliary dyskinesia.
The ciliary system defects are divided into primary and
secondary. The primary defects have a genetic origin.
Several genes have been identiﬁed, the mutations in
which lead, supposedly, to ciliary dyskinesia: those
encoding heavy (Bartoloni
, 2001; Olbrich
, 2002), intermediate (Zariwala
, 2001; Bartoloni
, 2001), and light (Susalka
, 2002) chains of axoneme dyneins. The secondary
defects comprise changes arisen as a result of bacterial
Here, we studied the ultrastructure of ciliary cells of
the bronchus epithelium in patients with Kartagener
syndrome. The aim of this work was to described cen-
triole formation and ciliogenesis in man in the case of
deﬁcient mobile activity of the ciliary system. The main
attention was paid to the studies of ciliary systems in
members of the same family.
Specific Features of Centriole Formation and Ciliogenesis
in Ciliary Epithelium Cells of Respiratory Tracts in Patients
with Kartagener Syndrome
K. E. Domaratskii*, E. V. Uvakina**, I. K. Volkov**, and G. E. Onishchenko*
* Moscow State University, Vorob’evy Gory, Moscow, 119992 Russia
** Scientiﬁc Center of Health, Research Institute of Pediatrics, Ministry of Health of the Russian Federation,
Lomonosovskii prosp. 2/62, Moscow, 199991 Russia
Received June 17, 2004; in ﬁnal form, November 1, 2004
—An electron microscopic study of the ciliary epithelium of respiratory tracts was carried out in chil-
dren (members of the same family) with Kartagener syndrome, which is a variant of ciliary dyskinesia. It was
shown that in the case of both mobile cilia and ciliary dyskinesia in man, centrioles are formed during formation
of the ciliary basal bodies predominantly
, involving deuterosomes. A wide spectrum of pathological
changes was described in literature, such as the absence of dynein arms in the axoneme and disorganization of
axoneme structure. In addition to these changes in the ciliary system, we found integration of several ciliary
axonemes by the same plasma membrane, running of microtubules from the plasma membrane as bundles, dif-
ferent orientation of basal legs, etc.
: Kartagener syndrome, ciliary dyskinesia, centriole, basal body, ciliogenesis, epithelium.