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Expression analysis of BMP2, BMP5, BMP10 in human colon tissues from Hirschsprung disease patients.
[hirschsprung disease]
Bone
morphogenetic
proteins
(
BMPs
)
are
members
of
the
transforming
growth
factor
β
(
TGF
β
)
superfamily
.
BMP
2
,
BMP
5
and
BMP
10
exert
their
biological
functions
by
interacting
with
membrane
bound
receptors
belonging
to
the
serine
/
threonine
kinase
family
.
Hirschsprung
disease
(
HSCR
)
is
characterized
by
the
absence
of
intramural
ganglion
cells
in
the
nerve
plexuses
of
the
distal
gut
.
However
,
putative
Notch
function
in
enteric
nervous
system
(
ENS
)
development
and
the
etiology
of
HSCR
is
unknown
.
Aganglionic
and
ganglionic
colon
segment
tissues
of
50
HSCR
patients
were
investigated
for
the
expression
pattern
of
BMP
2
,
BMP
5
and
BMP
10
using
real-time
RT-PCR
,
Western
blot
analysis
and
immunohistochemical
staining
.
The
mRNA
levels
of
BMP
2
,
BMP
5
and
BMP
10
in
the
stenotic
colon
segment
from
HSCR
patients
were
significantly
higher
than
those
in
the
normal
ones
.
Similar
increased
expressions
of
them
in
the
stenotic
colon
segments
were
detected
by
Western
blotting
coupled
with
densitometry
analysis
.
Lastly
,
immunohistologicl
stain
showed
significant
BMP
2
,
5
and
10
increases
in
mucous
and
muscular
layers
from
stenotic
colon
segments
compared
to
normal
segments
.
B
MP
2
,
BMP
5
and
BMP
10
are
elevated
in
the
stenotic
colon
segment
of
HSCR
,
and
BMPs
signaling
plays
a
pivotal
role
in
the
development
of
HSCR
.
Diseases
Validation
Diseases presenting
"growth factor β"
symptom
esophageal squamous cell carcinoma
hirschsprung disease
inclusion body myositis
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