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Reduced expression of dentin sialophosphoprotein is associated with dysplastic dentin in mice overexpressing transforming growth factor-beta 1 in teeth.
[dentin dysplasia]
Transforming
growth
factor
(
TGF
)
-
beta
1
is
expressed
in
developing
tooth
from
the
initiation
stage
through
adulthood
.
Odontoblast-
specific
expression
of
TGF-
beta
1
in
the
tooth
continues
throughout
life
;
however
,
the
precise
biological
functions
of
this
growth
factor
in
the
odontoblasts
are
not
clearly
understood
.
Herein
,
we
describe
the
generation
of
transgenic
mice
that
overexpress
active
TGF-
beta
1
predominantly
in
the
odontoblasts
.
Teeth
of
these
mice
show
a
significant
reduction
in
the
tooth
mineralization
,
defective
dentin
formation
,
and
a
relatively
high
branching
of
dentinal
tubules
.
Dentin
extracellular
matrix
components
such
as
type
I
and
III
collagens
are
increased
and
deposited
abnormally
in
the
dental
pulp
,
similar
to
the
hereditary
human
tooth
disorders
such
as
dentin
dysplasia
and
dentinogenesis
imperfecta
.
Calcium
,
one
of
the
crucial
inorganic
components
of
mineralization
,
is
also
apparently
increased
in
the
transgenic
mouse
teeth
.
Most
importantly
,
the
expression
of
dentin
sialophosphoprotein
(
dspp
)
,
a
candidate
gene
implicated
in
dentinogenesis
imperfecta
II
(
MIM
125420
)
,
is
significantly
down-regulated
in
the
transgenic
teeth
.
Our
results
provide
in
vivo
evidence
suggesting
that
TGF-
beta
1
mediated
expression
of
dspp
is
crucial
for
dentin
mineralization
.
These
findings
also
provide
for
the
first
time
a
direct
experimental
evidence
indicating
that
decreased
dspp
gene
expression
along
with
the
other
cellular
changes
in
odontoblasts
may
result
in
human
hereditary
dental
disorders
like
dentinogenesis
imperfecta
II
(
MIM
125420
)
and
dentin
dysplasia
(
MIM
125400
and
125420
)
.
Diseases
Validation
Diseases presenting
"precise biological functions of this growth factor in the odontoblasts"
symptom
dentin dysplasia
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