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Vascular accumulation of the small leucine-rich proteoglycan decorin in CADASIL.
[cadasil]
Small
penetrating
brain
artery
thickening
is
a
major
feature
of
cerebral
autosomal
dominant
arteriopathy
with
subcortical
infarcts
and
leukoencephalopathy
(
CADASIL
)
.
Although
affected
fibrotic
arteries
of
CADASIL
have
been
shown
to
accumulate
collagen
,
other
components
that
compose
pathological
arterial
walls
remain
incompletely
characterized
.
We
investigated
the
expression
of
decorin
(
DCN
)
,
the
first
collagen-binding
small
leucine-rich
proteoglycan
identified
,
in
CADASIL
.
DCN
was
markedly
upregulated
in
pathologically
affected
leptomeningeal
and
small
penetrating
arteries
in
CADASIL
and
was
notably
weaker
in
normal
arteries
from
control
brains
.
DCN
protein
was
localized
principally
to
the
media
and
adventitia
and
only
occasionally
expressed
in
the
intima
.
Immunoblotting
of
brain
lysates
showed
a
three-fold
increase
of
DCN
in
CADASIL
brains
(
compared
with
controls
)
.
Messenger
RNA
encoding
DCN
was
five-fold
increased
in
CADASIL
.
We
conclude
that
DCN
is
the
first
identified
proteoglycan
to
be
identified
in
CADASIL
arteries
and
may
accumulate
through
transcriptional
mechanisms
.
Additional
studies
are
warranted
to
determine
whether
DCN
localizes
broadly
to
pathological
small
vessels
in
other
cerebrovascular
disorders
.
Diseases
Validation
Diseases presenting
"small penetrating arteries in cadasil and was notably weaker in normal arteries from control brains"
symptom
cadasil
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