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
.