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Presence of intramucosal neuroglial cells in normal and aganglionic human colon.
[hirschsprung disease]
The
enteric
nervous
system
(
ENS
)
is
composed
of
neural
crest-derived
neurons
(
also
known
as
ganglion
cells
)
the
cell
bodies
of
which
are
located
in
the
submucosal
and
myenteric
plexuses
of
the
intestinal
wall
.
Intramucosal
ganglion
cells
are
known
to
exist
but
are
rare
and
often
considered
ectopic
.
Also
derived
from
the
neural
crest
are
enteric
glial
cells
that
populate
the
ganglia
and
the
associated
nerves
,
as
well
as
the
lamina
propria
of
the
intestinal
mucosa
.
In
Hirschsprung
disease
(
HSCR
)
,
ganglion
cells
are
absent
from
the
distal
gut
because
of
a
failure
of
neural
crest-derived
progenitor
cells
to
complete
their
rostrocaudal
migration
during
embryogenesis
.
The
fate
of
intramucosal
glial
cells
in
human
HSCR
is
essentially
unknown
.
We
demonstrate
a
network
of
intramucosal
cells
that
exhibit
dendritic
morphology
typical
of
neurons
and
glial
cells
.
These
dendritic
cells
are
present
throughout
the
human
gut
and
express
Tuj
1
,
S
100
,
glial
fibrillary
acidic
protein
,
CD
5
6
,
synaptophysin
,
and
calretinin
,
consistent
with
mixed
or
overlapping
neuroglial
differentiation
.
The
cells
are
present
in
aganglionic
colon
from
patients
with
HSCR
,
but
with
an
altered
immunophenotype
.
Coexpression
of
Tuj
1
and
HNK
1
in
this
cell
population
supports
a
neural
crest
origin
.
These
findings
extend
and
challenge
the
current
understanding
of
ENS
microanatomy
and
suggest
the
existence
of
an
intramucosal
population
of
neural
crest-derived
cells
,
present
in
HSCR
,
with
overlapping
immunophenotype
of
neurons
and
glia
.
Intramucosal
neuroglial
cells
have
not
been
previously
recognized
,
and
their
presence
in
HSCR
poses
new
questions
about
ENS
development
and
the
pathobiology
of
HSCR
that
merit
further
investigation
.
Diseases
Validation
Diseases presenting
"distal gut"
symptom
hirschsprung disease
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