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Cystatin C in Alzheimer's disease.
[hereditary cerebral hemorrhage with amyloidosis]
Changes
in
expression
and
secretion
levels
of
cystatin
C
(
CysC
)
in
the
brain
in
various
neurological
disorders
and
in
animal
models
of
neurodegeneration
underscore
a
role
for
CysC
in
these
conditions
.
A
polymorphism
in
the
CysC
gene
(
CST
3
)
is
linked
to
increased
risk
for
Alzheimer
's
disease
(
AD
)
.
AD
pathology
is
characterized
by
deposition
of
oligomeric
and
fibrillar
forms
of
amyloid
β
(
Aβ
)
in
the
neuropil
and
cerebral
vessel
walls
,
neurofibrillary
tangles
composed
mainly
of
hyperphosphorylated
tau
,
and
neurodegeneration
.
The
implication
of
CysC
in
AD
was
initially
suggested
by
its
co
-localization
with
Aβ
in
amyloid-laden
vascular
walls
,
and
in
senile
plaque
cores
of
amyloid
in
the
brains
of
patients
with
AD
,
Down
's
syndrome
,
hereditary
cerebral
hemorrhage
with
amyloidosis
,
Dutch
type
(
HCHWA-D
)
,
and
cerebral
infarction
.
CysC
also
co
-localizes
with
Aβ
amyloid
deposits
in
the
brains
of
non-demented
aged
individuals
.
Multiple
lines
of
research
show
that
CysC
plays
protective
roles
in
AD
.
In
vitro
studies
have
shown
that
CysC
binds
Aβ
and
inhibits
Aβ
oligomerization
and
fibril
formation
.
In
vivo
results
from
the
brains
and
plasma
of
Aβ-depositing
transgenic
mice
confirmed
the
association
of
CysC
with
the
soluble
,
non-pathological
form
of
Aβ
and
the
inhibition
of
Aβ
plaques
formation
.
The
association
of
CysC
with
Aβ
was
also
found
in
brain
and
in
cerebrospinal
fluid
(
CSF
)
from
AD
patients
and
non-demented
control
individuals
.
Moreover
,
in
vitro
results
showed
that
CysC
protects
neuronal
cells
from
a
variety
of
insults
that
may
cause
cell
death
,
including
cell
death
induced
by
oligomeric
and
fibrillar
Aβ
.
These
data
suggest
that
the
reduced
levels
of
CysC
manifested
in
AD
contribute
to
increased
neuronal
vulnerability
and
impaired
neuronal
ability
to
prevent
neurodegeneration
.
This
review
elaborates
on
the
neuroprotective
roles
of
CysC
in
AD
and
the
clinical
relevance
of
this
protein
as
a
therapeutic
agent
.
Diseases
Validation
Diseases presenting
"cerebral hemorrhage"
symptom
cohen syndrome
hereditary cerebral hemorrhage with amyloidosis
sneddon syndrome
This symptom has already been validated
