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Stem cell factor and granulocyte colony-stimulating factor exhibit therapeutic effects in a mouse model of CADASIL.
[cadasil]
Cerebral
autosomal
dominant
arteriopathy
with
subcortical
infarcts
and
leukoencephalopathy
(
CADASIL
)
,
a
Notch
3
dominant
mutation
-induced
cerebral
small
vascular
disease
,
is
characterized
by
progressive
degeneration
of
vascular
smooth
muscle
cells
(
vSMCs
)
of
small
arteries
in
the
brain
,
leading
to
recurrent
ischemic
stroke
,
vascular
dementia
and
death
.
To
date
,
no
treatment
can
stop
or
delay
the
progression
of
this
disease
.
Herein
,
we
determined
the
therapeutic
effects
of
stem
cell
factor
(
SCF
)
in
combination
with
granulocyte
colony-stimulating
factor
(
G-CSF
)
(
SCF
+
G-CSF
)
in
a
mouse
model
of
CADASIL
carrying
the
human
mutant
Notch
3
gene
.
SCF
+
G-CSF
was
subcutaneously
administered
for
5
days
and
repeated
4
times
with
1
-
4
month
intervals
.
We
found
through
water
maze
testing
that
SCF
+
G-CSF
treatment
improved
cognitive
function
.
SCF
+
G-CSF
also
attenuated
vSMC
degeneration
in
small
arteries
,
increased
cerebral
blood
vascular
density
,
and
inhibited
apoptosis
in
CADASIL
mice
.
We
also
discovered
that
loss
of
cerebral
capillary
endothelial
cells
and
neural
stem
cells
/
neural
progenitor
cells
(
NSCs
/
NPCs
)
occurred
in
CADASIL
mice
.
SCF
+
G-CSF
treatment
inhibited
the
CADASIL
-induced
cell
loss
in
the
endothelia
and
NSCs
/
NPCs
and
promoted
neurogenesis
.
In
an
in
vitro
model
of
apoptosis
,
SCF
+
G-CSF
prevented
apoptotic
cell
death
in
vSMCs
through
AKT
signaling
and
by
inhibiting
caspase-
3
activity
.
These
data
suggest
that
SCF
+
G-CSF
restricts
the
pathological
progression
of
CADASIL
.
This
study
offers
new
insights
into
developing
therapeutic
strategies
for
CADASIL
.
Diseases
Validation
Diseases presenting
"is characterized by progressive degeneration of vascular smooth muscle cells"
symptom
cadasil
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