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Small heat shock proteins associated with cerebral amyloid angiopathy of hereditary cerebral hemorrhage with amyloidosis (Dutch type) induce interleukin-6 secretion.
[hereditary cerebral hemorrhage with amyloidosis]
In
hereditary
cerebral
hemorrhage
with
amyloidosis
of
the
Dutch
type
(
HCHWA-D
)
,
severe
cerebral
amyloid
angiopathy
(
CAA
)
is
associated
with
an
inflammatory
reaction
.
Small
heat
shock
proteins
(
sHsps
)
are
molecular
chaperones
and
association
of
HspB
8
with
CAA
in
HCHWA-D
has
been
observed
.
The
aims
of
this
study
were
to
investigate
(
1
)
if
other
sHsps
are
associated
with
the
pathological
lesions
in
HCHWA-D
brains
,
(
2
)
if
the
amyloid-
beta
protein
(
A
beta
)
increases
production
of
sHsps
in
cultured
cerebral
cells
and
(
3
)
if
sHsps
are
involved
in
the
cerebral
inflammatory
processes
in
both
Alzheimer
's
disease
(
AD
)
and
HCHWA-D
.
We
conclude
that
Hsp
20
,
HspB
8
and
HspB
2
are
present
in
CAA
in
HCHWA-D
,
and
that
A
beta
did
not
affect
cellular
sHsps
expression
in
cultured
human
brain
pericytes
and
astrocytes
.
In
addition
,
we
demonstrated
that
Hsp
20
,
HspB
2
and
HspB
8
induced
interleukin-
6
production
in
cultured
pericytes
and
astrocytes
,
which
could
be
antagonized
by
dexamethasone
,
whereas
other
sHsps
and
A
beta
were
inactive
,
suggesting
that
sHsps
may
be
among
the
key
mediators
of
the
local
inflammatory
response
associated
with
HCHWA-D
and
AD
lesions
.
Diseases
Validation
Diseases presenting
"small heat shock proteins"
symptom
alexander disease
hereditary cerebral hemorrhage with amyloidosis
inclusion body myositis
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