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Cerebrovascular smooth muscle cell surface fibrillar A beta. Alteration of the proteolytic environment in the cerebral vessel wall.
[hereditary cerebral hemorrhage with amyloidosis]
Cerebrovascular
deposition
of
the
amyloid
beta
-protein
(
A
beta
)
is
a
common
pathologic
event
in
patients
with
Alzheimer
's
disease
(
AD
)
and
certain
related
disorders
including
hereditary
cerebral
hemorrhage
with
amyloidosis
Dutch
-
type
(
HCHWA-D
)
.
A
beta
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
A
beta
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
.
Recent
studies
from
our
laboratory
have
shown
that
cell-surface
assembly
of
A
beta
into
fibrillar
structures
causes
cellular
degeneration
via
an
apoptotic
pathway
and
creates
an
altered
proteolytic
microenvironment
on
the
cell
surface
of
human
cerebrovascular
smooth
muscle
cells
(
HCSM
cells
)
.
For
example
,
HCSM
cell-surface
A
beta
fibrils
serve
as
a
site
for
tight
binding
of
cell-secreted
amyloid
beta
-precursor
protein
(
A
beta
PP
)
.
Since
A
beta
PP
is
a
potent
inhibitor
of
key
proteinases
of
coagulation
cascade
,
its
enhanced
localization
on
the
A
beta
fibrils
would
provide
an
strong
anticoagulant
environment
.
In
addition
,
HCSM
cell-surface
A
beta
fibrils
are
potent
stimulators
of
tissue
plasminogen
activator
(
tPA
)
creating
a
profibrinolytic
milieu
.
Our
findings
indicate
that
A
beta
fibril
assembly
on
the
HCSM
cell
surface
causes
cellular
degeneration
and
results
in
both
a
strong
anticoagulant
and
fibrinolytic
environment
.
Together
,
these
altered
proteolytic
events
could
create
a
setting
that
is
conducive
to
loss
of
vessel
wall
integrity
and
hemorrhagic
stroke
.
Diseases
Validation
Diseases presenting
"medial layer of the cerebral vessel wall"
symptom
hereditary cerebral hemorrhage with amyloidosis
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