Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Apoptosis-inducing factor deficiency decreases the proliferation rate and protects the subventricular zone against ionizing radiation.
[lamellar ichthyosis]
Cranial
radiotherapy
in
children
often
leads
to
progressive
cognitive
decline
.
We
have
established
a
rodent
model
of
irradiation-induced
injury
to
the
young
brain
.
A
single
dose
of
8
Gy
was
administered
to
the
left
hemisphere
of
postnatal
day
10
(
P
10
)
mice
.
Harlequin
(
Hq
)
mice
,
carrying
the
hypomorphic
apoptosis-inducing
factor
AIF
(
Hq
)
mutation
,
express
60
%
less
AIF
at
P
10
and
displayed
significantly
fewer
dying
cells
in
the
subventricular
zone
(
SVZ
)
6
h
after
IR
,
compared
with
wild
type
(
Wt
)
littermates
.
Irradiated
cyclophilin
A-
deficient
(
CypA
(
-
/
-
)
)
mice
confirmed
that
CypA
has
an
essential
role
in
AIF-induced
apoptosis
after
IR
.
Hq
mice
displayed
no
reduction
in
SVZ
size
7
days
after
IR
,
whereas
48
%
of
the
SVZ
was
lost
in
Wt
mice
.
The
proliferation
rate
was
lower
in
the
SVZ
of
Hq
mice
.
Cultured
neural
precursor
cells
from
the
SVZ
of
Hq
mice
displayed
a
slower
proliferation
rate
and
were
more
resistant
to
IR
.
IR
preferentially
kills
proliferating
cells
,
and
the
slower
proliferation
rate
in
the
SVZ
of
Hq
mice
may
,
at
least
partly
,
explain
the
protective
effect
of
the
Hq
mutation
.
Together
,
these
results
indicate
that
targeting
AIF
may
provide
a
fruitful
strategy
for
protection
of
normal
brain
tissue
against
the
detrimental
side
effects
of
IR
.
Diseases
Validation
Diseases presenting
"postnatal day"
symptom
canavan disease
child syndrome
dentinogenesis imperfecta
hydrocephalus with stenosis of the aqueduct of sylvius
lamellar ichthyosis
pendred syndrome
zellweger syndrome
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom