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Ion channels, channelopathies, and tooth formation.
[dentin dysplasia]
The
biological
functions
of
ion
channels
in
tooth
development
vary
according
to
the
nature
of
their
gating
,
the
species
of
ions
passing
through
those
gates
,
the
number
of
gates
,
localization
of
channels
,
tissue
expressing
the
channel
,
and
interactions
between
cells
and
microenvironment
.
Ion
channels
feature
unique
and
specific
ion
flux
in
ameloblasts
,
odontoblasts
,
and
other
tooth-
specific
cell
lineages
.
Both
enamel
and
dentin
have
active
chemical
systems
orchestrating
a
variety
of
ion
exchanges
and
demineralization
and
remineralization
processes
in
a
stage
-dependent
manner
.
An
important
role
for
ion
channels
is
to
regulate
and
maintain
the
calcium
and
pH
homeostasis
that
are
critical
for
proper
enamel
and
dentin
biomineralization
.
Specific
functions
of
chloride
channels
,
TRPVs
,
calcium
channels
,
potassium
channels
,
and
solute
carrier
superfamily
members
in
tooth
formation
have
been
gradually
clarified
in
recent
years
.
Mutations
in
these
ion
channels
or
transporters
often
result
in
disastrous
changes
in
tooth
development
.
The
channelopathies
of
tooth
include
altered
eruption
(
CLCN
7
,
KCNJ
2
,
TRPV
3
)
,
root
dysplasia
(
CLCN
7
,
KCNJ
2
)
,
amelogenesis
imperfecta
(
KCNJ
1
,
CFTR
,
AE
2
,
CACNA
1
C
,
GJA
1
)
,
dentin
dysplasia
(
CLCN
5
)
,
small
teeth
(
CACNA
1
C
,
GJA
1
)
,
tooth
agenesis
(
CLCN
7
)
,
and
other
impairments
.
The
mechanisms
leading
to
tooth
channelopathies
are
primarily
related
to
pH
regulation
,
calcium
homeostasis
,
or
other
alterations
of
the
niche
for
tooth
eruption
and
development
.
Diseases
Validation
Diseases presenting
"or other alterations of the niche for tooth eruption and development"
symptom
dentin dysplasia
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