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Mandibular phenotype of p20C/EBPbeta transgenic mice: Reduced alveolar bone mass and site-specific dentin dysplasia.
[dentin dysplasia]
CCAAT
enhancer
binding
proteins
(
C
/
EBP
)
comprise
a
family
of
basic-leucine
zipper
transcription
factors
that
regulate
cellular
differentiation
and
function
.
To
determine
the
role
of
C
/
EBP
transcription
factors
in
osteoblasts
and
odontoblasts
,
we
generated
a
transgenic
(
TG
)
mouse
model
with
Co
1
a
1
(
pOBCol
3
.
6
)
promoter-targeted
expression
of
a
FLAG-tagged
dominant
negative
C
/
EBP
isoform
,
p
20
C
/
EBPbeta
(
previously
LIP
)
.
Two
of
the
four
transgenic
lines
presented
with
abnormalities
in
the
developing
incisors
,
including
breakage
,
overgrowth
,
and
malocclusion
.
Histological
examination
revealed
that
the
amount
of
alveolar
bone
was
reduced
in
TG
compared
to
wild-
type
(
WT
)
mice
.
By
microcomputed
tomography
(
microCT
)
,
the
bone
volume
fraction
of
the
mandible
was
reduced
at
the
level
of
the
first
and
third
molars
,
demonstrating
a
severe
mandibular
osteopenia
.
The
lingual
dentin
morphology
of
TG
incisors
differed
dramatically
from
WT
.
Labial
dentin
(
enamel
side
)
showed
normal
thickness
and
tubular
dentin
structure
,
whereas
the
lingual
dentin
was
thinner
(
25
-
30
%
of
WT
at
the
alveolar
crest
)
with
an
amorphous
globular
structure
characteristic
of
dentin
dysplasia
.
FLAG
immunostaining
was
seen
in
both
lingual
and
labial
odontoblasts
,
indicating
that
the
site-
specific
defect
was
not
due
to
a
lack
of
labial
transgene
expression
.
Northern
blot
analysis
demonstrated
reduced
osteocalcin
expression
in
TG
mandibles
,
while
bone
sialoprotein
was
increased
,
consistent
with
prior
results
in
calvariae
and
long
bones
.
Dental
sialophosphoprotein
,
a
marker
of
the
odontoblast
lineage
whose
absence
causes
dentin
dysplasia
,
was
modestly
reduced
in
TG
mice
by
Northern
blot
and
in
situ
hybridization
analysis
.
By
fluorescence
microscopy
,
pOBCol
2
.
3
-
GFP
,
a
marker
of
the
odontoblast
lineage
,
was
expressed
in
both
labial
and
lingual
odontoblasts
,
although
GFP-
marked
lingual
odontoblasts
were
more
flattened
than
WT
cells
.
Moreover
,
GFP-
positive
processes
in
the
lingual
dentin
tubules
were
truncated
and
less
organized
than
those
in
WT
dentin
.
MicroCT
analysis
showed
reduced
tissue
density
in
the
lingual
dentin
.
These
data
suggest
that
C
/
EBP
transcription
factors
may
be
involved
in
the
regulation
of
odontoblast
polarization
and
dentin
matrix
production
.
Diseases
Validation
Diseases presenting
"tubular dentin structure"
symptom
dentin dysplasia
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