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Genetic evidence for key roles of decorin and biglycan in dentin mineralization.
[dentinogenesis imperfecta]
Targeted
disruption
of
the
dentin
sialophosphoprotein
(
DSPP
)
gene
in
the
mice
(
Dspp
(
-
/
-
)
)
results
in
dentin
mineralization
defects
with
enlarged
predentin
phenotype
similar
to
human
dentinogenesis
imperfecta
type
III
.
Using
DSPP
/
biglycan
(
Dspp
(
-
/
-
)
Bgn
(
-
/
0
)
)
and
DSPP
/
decorin
(
Dspp
(
-
/
-
)
Dcn
(
-
/
-
)
)
double
knockout
mice
,
here
we
determined
that
the
enlarged
predentin
layer
in
Dspp
(
-
/
-
)
teeth
is
rescued
in
the
absence
of
decorin
,
but
not
in
the
absence
of
biglycan
.
However
,
Fourier
transform
infrared
(
FTIR
)
spectroscopy
analysis
reveals
similar
hypomineralization
of
dentin
in
both
Dspp
(
-
/
-
)
Bgn
(
-
/
0
)
and
Dspp
(
-
/
-
)
Dcn
(
-
/
-
)
teeth
.
Atomic
force
microscopy
(
AFM
)
analysis
of
collagen
fibrils
in
dentin
shows
subtle
differences
in
the
collagen
fibril
morphology
in
these
genotypes
.
The
reduction
of
enlarged
predentin
in
Dspp
(
-
/
-
)
Dcn
(
-
/
-
)
mice
suggests
that
the
elevated
level
of
decorin
in
Dspp
(
-
/
-
)
predentin
interferes
with
the
mineralization
process
at
the
dentin
mineralization
front
.
On
the
other
hand
,
the
lack
of
DSPP
and
biglycan
leads
to
the
increased
number
of
calcospherites
in
Dspp
(
-
/
-
)
Bgn
(
-
/
0
)
predentin
,
suggesting
that
a
failure
in
coalescence
of
calcospherites
was
augmented
in
Dspp
(
-
/
-
)
Bgn
(
-
/
0
)
teeth
as
compared
to
Dspp
(
-
/
-
)
teeth
.
These
findings
indicate
that
normal
expression
of
small
leucine
rich
proteoglycans
,
such
as
biglycan
and
decorin
,
plays
an
important
role
in
the
highly
orchestrated
process
of
dentin
mineralization
.
Diseases
Validation
Diseases presenting
"absence of decorin"
symptom
dentinogenesis imperfecta
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