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Immunolocalisation of PDGFRβ and pericytes in CADASIL.
[cadasil]
Cerebral
autosomal
dominant
arteriopathy
with
subcortical
infarcts
and
leukoencephalopathy
(
CADASIL
)
is
identified
by
aggregates
of
NOTCH
3
extracellular
domain
(
N
3
ECD
)
along
capillaries
and
the
deposition
of
granular
osmiophilic
material
(
GOM
)
.
We
assessed
the
pattern
of
distribution
of
pericytes
in
relation
to
N
3
ECD
deposits
in
cerebral
microvessels
of
CADASIL
subjects
.
We
assessed
post-mortem
brains
from
(
n
=
50
)
subjects
with
CADASIL
,
cerebral
small
vessel
disease
and
similar
age
cognitively
normal
and
older
controls
.
Immunohistochemical
and
immunofluorescent
staining
methods
were
used
to
study
the
distribution
and
quantify
immunoreactivities
of
the
platelet
derived
growth
factor
receptor-β
(
PDGFR-β
)
(
for
pericytes
)
and
microvascular
markers
in
the
frontal
cortex
and
white
matter
.
P
DGFR-β
antibody
stained
cells
typical
of
pericytes
in
capillaries
and
small
arterioles
in
both
the
grey
and
white
matter
.
PDGFR-β
reactive
pericytes
adopted
"
crescent
"
morphology
wrapped
closely
around
capillary
walls
readily
evident
in
cross-sections
.
We
noted
considerable
overlap
between
PDGFR-β
and
N
3
ECD
imunoreactivities
in
capillaries
.
Quantitative
analysis
of
PDGFR-β
immunoreactivity
revealed
significant
differences
in
PDGFR-β
%
Area
in
CADASIL
compared
to
young
controls
(
P
<
0
.
05
)
.
PDGFR-β
percent
Area
was
further
positively
correlated
with
the
basement
membrane
marker
collagen
IV
(
r
=
0
.
529
,
P
=
0
.
009
)
,
but
was
not
associated
with
GLUT-
1
,
the
marker
for
endothelial
cells
.
Our
results
suggest
increased
expression
of
PDGFR-β
immunoreactive
pericytes
in
cerebral
microvessels
in
CADASIL
compared
to
similar
age
controls
.
While
we
can
not
confirm
whether
PDGFR-β-expressing
pericytes
produce
N
3
ECD
and
hence
GOM
,
our
findings
demonstrate
that
upregulation
of
pericyte-like
cells
is
associated
with
microvascular
changes
including
loss
of
vascular
smooth
muscle
cells
in
CADASIL
.
Diseases
Validation
Diseases presenting
"similar age cognitively normal and older controls"
symptom
cadasil
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