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Lipoprotein receptor-related protein-1 mediates amyloid-beta-mediated cell death of cerebrovascular cells.
[hereditary cerebral hemorrhage with amyloidosis]
Inefficient
clearance
of
A
beta
,
caused
by
impaired
blood
-
brain
barrier
crossing
into
the
circulation
,
seems
to
be
a
major
cause
of
A
beta
accumulation
in
the
brain
of
late-onset
Alzheimer
's
disease
patients
and
hereditary
cerebral
hemorrhage
with
amyloidosis
Dutch
type
.
We
observed
association
of
receptor
for
advanced
glycation
end
products
,
CD
36
,
and
low
-density
lipoprotein
receptor
(
LDLR
)
with
cerebral
amyloid
angiopathy
in
both
Alzheimer
's
disease
and
hereditary
cerebral
hemorrhage
with
amyloidosis
Dutch
type
brains
and
increased
low
-density
lipoprotein
receptor-related
protein-
1
(
LRP-
1
)
expression
by
perivascular
cells
in
cerebral
amyloid
angiopathy
.
We
investigated
if
these
A
beta
receptors
are
involved
in
A
beta
internalization
and
in
A
beta
-mediated
cell
death
of
human
cerebrovascular
cells
and
astrocytes
.
Expression
of
both
the
LRP-
1
and
LDLR
by
human
brain
pericytes
and
leptomeningeal
smooth
muscle
cells
,
but
not
by
astrocytes
,
increased
on
incubation
with
A
beta
.
Receptor-associated
protein
specifically
inhibited
A
beta
-mediated
up-regulation
of
LRP-
1
,
but
not
of
LDLR
,
and
receptor-associated
protein
also
decreased
A
beta
internalization
and
A
beta
-mediated
cell
death
.
We
conclude
that
especially
LRP-
1
and
,
to
a
minor
extent
,
LDLR
are
involved
in
A
beta
internalization
by
and
A
beta
-mediated
cell
death
of
cerebral
perivascular
cells
.
Although
perivascular
cells
may
adapt
their
A
beta
internalization
capacity
to
the
levels
of
A
beta
present
,
saturated
LRP-
1
/
LDLR
-mediated
uptake
of
A
beta
results
in
degeneration
of
perivascular
cells
.
Diseases
Validation
Diseases presenting
"cerebral hemorrhage"
symptom
cohen syndrome
hereditary cerebral hemorrhage with amyloidosis
sneddon syndrome
This symptom has already been validated