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Co-localization of beta/A4 and cystatin C in cortical blood vessels in Dutch, but not in Icelandic hereditary cerebral hemorrhage with amyloidosis.
[hereditary cerebral hemorrhage with amyloidosis]
Based
on
the
recent
discovery
of
co
-localization
of
beta
/
A
4
and
cystatin
C
in
cortical
blood
vessels
of
patients
with
cerebral
hemorrhages
due
to
sporadic
amyloid
angiopathy
and
patients
with
Alzheimer
's
disease
we
investigated
the
presence
of
these
two
proteins
in
the
cortical
blood
vessels
of
patients
suffering
from
hereditary
cerebral
hemorrhage
with
amyloidosis
of
the
Dutch
(
n
=
11
)
and
the
Icelandic
(
n
=
2
)
type
.
The
brains
of
three
patients
with
sporadic
cerebral
amyloid
angiopathy
were
also
investigated
.
Blood
vessels
of
the
Dutch
patients
clearly
showed
immunostaining
with
beta
/
A
4
as
well
as
with
cystatin
C
antibodies
,
whereas
the
blood
vessels
of
Icelandic
patients
showed
only
staining
with
cystatin
C
.
In
one
of
the
three
sporadic
amyloid
angiopathy
patients
co
-localization
was
shown
as
well
.
The
co
-localization
of
mutated
beta
/
A
4
with
normal
cystatin
C
in
the
Dutch
patients
suggests
that
cystatin
C
deposition
occurs
secondarily
to
beta
/
A
4
deposition
.
This
is
probably
also
the
case
in
sporadic
amyloid
angiopathy
and
Alzheimer
's
disease
.
Cystatin
C
deposition
may
play
a
role
in
the
development
of
cerebral
hemorrhages
and
leukoencephalopathy
.
Diseases
Validation
Diseases presenting
"blood vessels of the dutch patients"
symptom
hereditary cerebral hemorrhage with amyloidosis
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