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Enhanced Abeta40 deposition was associated with increased Abeta42-43 in cerebral vasculature with Dutch-type hereditary cerebral hemorrhage with amyloidosis (HCHWA-D).
[hereditary cerebral hemorrhage with amyloidosis]
Cerebrovascular
deposition
of
the
amyloid
beta
-protein
(
Abeta
)
is
a
common
pathologic
event
in
patients
with
Alzheimer
's
disease
(
AD
)
and
certain
related
disorders
.
Such
an
Abeta
vascular
deposition
occurs
primarily
in
the
medial
layer
of
the
cerebral
vessel
wall
in
an
assembled
fibrillar
state
.
These
deposits
are
associated
with
several
pathological
responses
,
including
degeneration
of
the
smooth
muscle
cells
in
the
cerebral
vessel
wall
.
Severe
cases
of
cerebrovascular
Abeta
deposition
are
also
accompanied
by
loss
of
vessel
wall
integrity
and
hemorrhagic
stroke
.
Although
the
reasons
for
this
pathological
consequence
are
unclear
,
altered
proteolytic
mechanisms
within
the
cerebral
vessel
wall
may
be
involved
.
We
analyzed
cerebral
Abeta
deposition
in
brains
with
AD
and
Dutch
-
type
hereditary
cerebral
hemorrhage
with
amyloidosis
(
HCHWA-D
)
on
the
basis
of
two
amyloid
species
of
Abeta
(
40
)
and
Abeta
(
42
/
43
)
using
specific
monoclonal
antibodies
.
Compared
to
Abeta
deposition
in
senile
plaques
,
the
molecular
composition
of
Abeta
was
distinguishable
,
indicating
that
the
Abeta
(
40
)
species
is
the
main
component
for
vascular
amyloid
.
Furthermore
,
we
found
Abeta
(
42
/
43
)
immunoreactivity
was
also
much
increased
in
amyloid
angiopathy
of
all
cases
with
HCHWA-D
.
Taken
together
,
amyloid
angiopathy
in
HCHWA-D
may
share
an
Abeta
(
42
)
-
driven
deposition
mechanism
with
plaque
amyloid
,
resulting
in
enhanced
Abeta
(
40
)
deposition
.
Diseases
Validation
Diseases presenting
"vascular amyloid"
symptom
hereditary cerebral hemorrhage with amyloidosis
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