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The role of cystatin C in cerebral amyloid angiopathy and stroke: cell biology and animal models.
[hereditary cerebral hemorrhage with amyloidosis]
A
variant
of
the
cysteine
protease
inhibitor
,
cystatin
C
,
forms
amyloid
deposited
in
the
cerebral
vasculature
of
patients
with
hereditary
cerebral
hemorrhage
with
amyloidosis
,
Icelandic
type
(
HCHWA
-
I
)
,
leading
to
cerebral
hemorrhages
early
in
life
.
However
,
cystatin
C
is
also
implicated
in
neuronal
degenerative
diseases
in
which
it
does
not
form
the
amyloid
protein
,
such
as
Alzheimer
disease
(
AD
)
.
Accumulating
data
suggest
involvement
of
cystatin
C
in
the
pathogenic
processes
leading
to
amyloid
deposition
in
cerebral
vasculature
and
most
significantly
to
cerebral
hemorrhage
in
patients
with
cerebral
amyloid
angiopathy
(
CAA
)
.
This
review
focuses
on
cell
culture
and
animal
models
used
to
study
the
role
of
cystatin
C
in
these
processes
.