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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
"osteopenia"
symptom
achondroplasia
aromatase deficiency
benign recurrent intrahepatic cholestasis
child syndrome
coats disease
cutaneous mastocytosis
dentin dysplasia
dentinogenesis imperfecta
erythropoietic protoporphyria
fabry disease
familial hypocalciuric hypercalcemia
This symptom has already been validated