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Role of the subcommissural organ in the pathogenesis of congenital hydrocephalus in the HTx rat.
[hydrocephalus with stenosis of the aqueduct of sylvius]
The
present
investigation
was
designed
to
clarify
the
role
of
the
subcommissural
organ
(
SCO
)
in
the
pathogenesis
of
hydrocephalus
occurring
in
the
HTx
rat
.
The
brains
of
non-affected
and
hydrocephalic
HTx
rats
from
embryonic
day
15
(
E
15
)
to
postnatal
day
10
(
PN
10
)
were
processed
for
electron
microscopy
,
lectin
binding
and
immunocytochemistry
by
using
a
series
of
antibodies
.
Cerebrospinal
fluid
(
CSF
)
samples
of
non-affected
and
hydrocephalic
HTx
rats
were
collected
at
PN
1
,
PN
7
and
PN
30
and
analysed
by
one
-
and
two
-dimensional
electrophoresis
,
immunoblotting
and
nanoLC-
ESI
-
MS
/
MS
.
A
distinct
malformation
of
the
SCO
is
present
as
early
as
E
15
.
Since
stenosis
of
the
Sylvius
aqueduct
(
SA
)
occurs
at
E
18
and
dilation
of
the
lateral
ventricles
starts
at
E
19
,
the
malformation
of
the
SCO
clearly
precedes
the
onset
of
hydrocephalus
.
In
the
affected
rats
,
the
cephalic
and
caudal
thirds
of
the
SCO
showed
high
secretory
activity
with
all
methods
used
,
whereas
the
middle
third
showed
no
signs
of
secretion
.
At
E
18
,
the
middle
non-secretory
third
of
the
SCO
progressively
fused
with
the
ventral
wall
of
SA
,
resulting
in
marked
aqueduct
stenosis
and
severe
hydrocephalus
.
The
abnormal
development
of
the
SCO
resulted
in
the
permanent
absence
of
Reissner
's
fibre
(
RF
)
and
led
to
changes
in
the
protein
composition
of
the
CSF
.
Since
the
SCO
is
the
source
of
a
large
mass
of
sialilated
glycoproteins
that
form
the
RF
and
of
those
that
remain
CSF-soluble
,
we
hypothesize
that
the
absence
of
this
large
mass
of
negatively
charged
molecules
from
the
SA
domain
results
in
SA
stenosis
and
impairs
the
bulk
flow
of
CSF
through
the
aqueduct
.
