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Myogenin gene expression is not altered in the developing diaphragm of nitrofen-induced congenital diaphragmatic hernia.
[congenital diaphragmatic hernia]
Pleuroperitoneal
folds
(
PPFs
)
represent
the
only
source
of
muscle
precursors
cells
(
MPCs
)
in
the
primordial
diaphragm
.
However
,
the
exact
pathogenesis
of
malformed
PPFs
and
congenital
diaphragmatic
hernia
(
CDH
)
remains
unclear
.
The
muscle
-
specific
transcription
factor
myogenin
plays
a
key
role
during
development
and
muscularization
of
the
fetal
diaphragm
.
Although
myogenin
knockout
mice
lack
skeletal
muscle
fibers
,
the
diaphragmatic
musculature
is
intact
without
any
defects
.
It
has
further
been
demonstrated
that
proliferation
and
differentiation
of
MPCs
in
PPFs
and
developing
diaphragms
are
normal
in
rodent
CDH
models
.
We
hypothesized
that
myogenin
gene
expression
is
not
altered
in
malformed
PPFs
,
developing
diaphragms
and
diaphragmatic
musculature
in
the
nitrofen-induced
CDH
model
.
Pregnant
rats
were
exposed
to
nitrofen
or
vehicle
on
gestational
day
9
(
D
9
)
.
Fetuses
were
harvested
during
PPF
formation
(
D
13
)
,
diaphragmatic
development
(
D
14
-
15
)
and
muscularization
(
D
18
-
21
)
.
Fetal
PPFs
,
developing
diaphragms
and
diaphragmatic
musculature
were
dissected
and
divided
into
nitrofen
and
control
groups
.
Myogenin
mRNA
levels
were
analyzed
by
quantitative
real-time
polymerase
chain
reaction
,
while
immunohistochemistry
was
performed
to
investigate
myogenin
protein
expression
and
distribution
.
Relative
mRNA
expression
of
myogenin
was
not
significant
different
in
PPFs
(
0
.
30
Â
±
Â
0
.
09
vs
.
0
.
48
Â
±
Â
0
.
09
;
P
Â
=
Â
0
.
37
)
,
developing
diaphragms
(
1
.
25
Â
±
Â
0
.
29
vs
.
1
.
60
Â
±
Â
0
.
32
;
P
=
0
.
53
)
and
diaphragmatic
musculature
(
1
.
08
Â
±
Â
0
.
24
vs
.
1
.
59
Â
±
Â
0
.
20
;
P
=
0
.
15
)
of
nitrofen-exposed
fetuses
compared
to
controls
.
Myogenin
immunoreactivity
was
not
altered
in
the
muscular
components
of
malformed
PPFs
,
developing
diaphragms
and
diaphragmatic
musculature
of
nitrofen-exposed
fetuses
compared
to
controls
.
Myogenin
gene
expression
is
not
altered
in
PPFs
,
developing
diaphragms
and
diaphragmatic
musculature
in
the
nitrofen-induced
CDH
model
,
thus
suggesting
that
diaphragmatic
defects
in
this
model
develop
independent
of
myogenic
processes
.
Diseases
Validation
Diseases presenting
"lack skeletal muscle fibers"
symptom
congenital diaphragmatic hernia
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