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Cranial suture biology and dental development: genetic and clinical perspectives.
[dentin dysplasia]
Premature
fusion
of
the
calvarial
bones
at
the
sutures
,
or
craniosynostosis
(
CS
)
,
is
a
relatively
common
birth
defect
(
1
:
2000
-
3000
)
frequently
associated
with
limb
deformity
.
Patients
with
CS
may
present
oral
defects
,
such
as
cleft
soft
palate
,
hypodontia
,
hyperdontia
,
and
delayed
tooth
eruption
,
but
also
unusual
associations
of
major
dental
anomalies
such
as
taurodontism
,
microdontia
,
multiple
dens
invaginatus
,
and
dentin
dysplasia
.
The
list
of
genes
that
are
involved
in
CS
includes
those
coding
for
the
different
fibroblast
growth
factor
receptors
and
a
ligand
of
ephrin
receptors
,
but
also
genes
encoding
transcription
factors
,
such
as
MSX
2
and
TWIST
.
Most
of
these
genes
are
equally
involved
in
odontogenesis
,
providing
a
pausible
explanation
for
clinical
associations
of
CS
with
dental
agenesis
or
tooth
malformations
.
On
the
basis
of
the
present
knowledge
on
genes
and
transcription
factors
that
are
involved
in
craniofacial
morphogenesis
,
and
from
dental
clinics
of
CS
syndromes
,
the
molecular
mechanisms
that
control
suture
formation
and
suture
closure
are
expected
to
play
key
roles
in
patterning
events
and
development
of
teeth
.
The
purpose
of
this
article
is
to
review
and
merge
the
recent
advances
in
the
field
of
suture
research
at
the
genetic
and
cellular
levels
with
those
of
tooth
development
,
and
to
apply
them
to
the
dental
clinics
of
CS
syndromes
.
These
new
perspectives
and
future
challenges
in
the
field
of
both
dental
clinics
and
molecular
genetics
,
more
in
particular
the
identification
of
possible
candidate
genes
involved
in
both
CS
and
dental
defects
,
are
discussed
.
Diseases
Validation
Diseases presenting
"craniofacial morphogenesis"
symptom
dentin dysplasia
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