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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
"growth factor"
symptom
22q11.2 deletion syndrome
achondroplasia
adrenal incidentaloma
aniridia
cadasil
cholangiocarcinoma
coats disease
dedifferentiated liposarcoma
dentin dysplasia
dentinogenesis imperfecta
dystrophic epidermolysis bullosa
esophageal carcinoma
esophageal squamous cell carcinoma
hodgkin lymphoma, classical
holt-oram syndrome
inclusion body myositis
kallmann syndrome
krabbe disease
liposarcoma
lymphangioleiomyomatosis
oculocutaneous albinism
oral submucous fibrosis
pleomorphic liposarcoma
primary effusion lymphoma
primary hyperoxaluria type 1
severe combined immunodeficiency
systemic capillary leak syndrome
von hippel-lindau disease
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
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