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Effects of lipopolysaccharide-induced inflammation on the interstitial cells of Cajal.
[hirschsprung disease]
Interstitial
cells
of
Cajal
(
ICC
)
have
recently
been
found
to
display
phenotypic
changes
.
The
present
study
is
designed
to
determine
whether
phenotypic
changes
occur
in
ICC
associated
with
an
inflammatory
microenvironment
and
whether
the
ICC
phenotype
could
be
recovered
after
the
discontinuation
of
inflammatory
stimuli
.
Immunohistochemistry
studies
revealed
that
the
functional
ICC
marker
,
c-kit
,
was
markedly
reduced
in
patients
with
Hirschsprung
's
disease
(
n
=
34
)
compared
with
controls
(
n
=
12
)
,
whereas
another
marker
of
ICC
,
CD
34
,
was
not
altered
significantly
.
Compared
with
the
vehicle
group
(
n
=
6
)
,
intraperitoneal
injection
of
lipopolysaccharide
(
LPS
;
1
.
5
mg
/
kg
)
in
mice
(
n
=
6
)
significantly
induced
plasma
tumor
necrosis
factor
-alpha
(
TNF
-α
)
levels
as
determined
by
enzyme-linked
immunosorbent
assay
.
Western
blot
and
real-time
polymerase
chain
reaction
assessment
further
showed
that
LPS
injection
markedly
suppressed
intestinal
c-kit
protein
and
mRNA
expression
,
which
could
be
blocked
by
Toll-like
receptor
4
(
TLR
4
)
deficiency
(
n
=
6
)
rather
than
TLR
2
deficiency
(
n
=
6
)
and
had
no
effects
on
CD
34
.
Compared
with
the
vehicle
group
(
n
=
6
)
,
intraperitoneal
TNF
-α
(
30
μg
/
kg
)
administration
(
n
=
6
)
also
significantly
reduced
intestinal
c-kit
protein
and
mRNA
levels
but
not
CD
34
levels
.
However
,
the
reduction
of
c-kit
induced
by
TNF
-α
injection
was
not
suppressed
by
TLR
4
deficiency
(
n
=
6
)
.
Intestinal
c-kit
protein
and
mRNA
levels
were
markedly
restored
after
the
discontinuation
of
TNF
-α
administration
for
7
days
.
Moreover
,
immunofluorescence
analysis
of
primary
ICC
further
confirmed
that
exposure
to
TNF
-α
for
24
h
suppressed
c-kit
expression
,
which
could
be
restored
after
discontinuation
of
TNF
-α
exposure
.
CD
34
expression
was
not
altered
upon
exposure
to
TNF
-α
.
Thus
,
phenotypic
changes
in
ICC
occur
in
an
inflammatory
microenvironment
in
the
gut
and
LPS
,
TLR
4
and
TNF
α
are
crucial
to
this
process
.
Diseases
Validation
Diseases presenting
"lps"
symptom
hirschsprung disease
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