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Cyclophilin D as a potential target for antioxidants in neurodegeneration: the X-ALD case.
[x-linked adrenoleukodystrophy]
X-
linked
adrenoleukodystrophy
(
X-
ALD
)
is
a
severe
inherited
neurodegenerative
disorder
characterized
by
adrenal
insufficiency
and
graded
damage
in
the
nervous
system
.
Loss
of
function
of
the
peroxisomal
ABCD
1
fatty-acid
transporter
,
resulting
in
the
accumulation
of
very
long
-chain
fatty
acids
in
organs
and
plasma
,
is
the
genetic
cause
.
Treatment
with
a
combination
of
antioxidants
halts
the
axonal
degeneration
and
locomotor
impairment
displayed
by
the
animal
model
of
X-
ALD
,
and
is
a
proof
of
concept
that
oxidative
stress
contributes
to
axonal
damage
.
New
evidence
demonstrates
that
metabolic
failure
and
the
opening
of
the
mitochondrial
permeability
transition
pore
orchestrated
by
cyclophilin
D
underlies
oxidative
stress-induced
axonal
degeneration
.
Thus
,
cyclophilin
D
could
serve
as
a
therapeutic
target
for
the
treatment
of
X-
ALD
and
cyclophilin
D-
dependent
neurodegenerative
and
non-neurodegenerative
diseases
.
