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Characterization of interstitial Cajal progenitors cells and their changes in Hirschsprung's disease.
[hirschsprung disease]
Interstitial
cells
of
Cajal
(
ICC
)
are
critical
to
gastrointestinal
motility
.
The
phenotypes
of
ICC
progenitors
have
been
observed
in
the
mouse
gut
,
but
whether
they
exist
in
the
human
colon
and
what
abnormal
changes
in
their
quantity
and
ultrastructure
are
present
in
Hirschsprung
's
disease
(
HSCR
)
colon
remains
uncertain
.
In
this
study
,
we
collected
the
surgical
resection
of
colons
,
both
proximal
and
narrow
segments
,
from
HSCR
patients
and
normal
controls
.
First
,
we
identified
the
progenitor
of
ICC
in
normal
adult
colon
using
immunofluorescent
localization
techniques
with
laser
confocal
microscopy
.
Next
,
the
progenitors
were
sorted
to
observe
their
morphology
.
We
further
applied
flow
cytometry
to
examine
the
content
of
ICC
progenitors
in
these
fresh
samples
.
The
ultrastructural
changes
in
the
narrow
and
proximal
parts
of
the
HSCR
colon
were
observed
using
transmission
electron
microscopy
(
TEM
)
and
were
compared
with
the
normal
adult
colon
.
The
presumed
early
progenitor
(
c-
Kit
(
low
)
CD
34
(
+
)
Igf
1
r
(
+
)
)
and
committed
progenitor
(
c-
Kit
(
+
)
CD
34
(
+
)
Igf
1
r
(
+
)
)
of
ICC
exist
in
adult
normal
colon
as
well
as
in
the
narrow
and
proximal
parts
of
the
HSCR
colon
.
However
,
the
proportions
of
mature
,
early
and
committed
progenitors
of
ICC
were
dramatically
reduced
in
the
narrow
segment
of
the
HSCR
colon
.
The
proportions
of
mature
and
committed
progenitors
of
ICC
in
the
proximal
segment
of
the
HSCR
colon
were
lower
than
in
the
adult
normal
colon
.
Ultrastructurally
,
ICC
,
enteric
nerves
,
and
smooth
muscle
in
the
narrow
segment
of
the
HSCR
colon
showed
severe
injury
,
including
swollen
vacuola
or
ted
mitochondria
,
disappearance
of
mitochondrial
cristae
,
dilated
rough
endoplasmic
reticulum
,
vesiculation
and
degranulation
,
and
disappearance
of
the
caveolae
on
the
ICC
membrane
surface
.
The
contents
of
ICC
and
its
progenitors
in
the
narrow
part
of
the
HSCR
colon
were
significantly
decreased
than
those
of
adult
colon
,
which
may
be
associated
with
HSCR
pathogenesis
.
Diseases
Validation
Diseases presenting
"narrow segments"
symptom
hirschsprung disease
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