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Oxidative stress regulates the ubiquitin-proteasome system and immunoproteasome functioning in a mouse model of X-adrenoleukodystrophy.
[x-linked adrenoleukodystrophy]
Oxidative
damage
is
a
pivotal
aetiopathogenic
factor
in
X-
linked
adrenoleukodystrophy
.
This
is
a
neurometabolic
disease
characterized
by
the
accumulation
of
very
-
long
-chain
fatty
acids
owing
to
the
loss
of
function
of
the
peroxisomal
transporter
Abcd
1
.
Here
,
we
used
the
X-
linked
adrenoleukodystrophy
mouse
model
and
patient
's
fibroblasts
to
detect
malfunctioning
of
the
ubiquitin-proteasome
system
resulting
from
the
accumulation
of
oxidatively
modified
proteins
,
some
involved
in
bioenergetic
metabolism
.
Furthermore
,
the
immunoproteasome
machinery
appears
upregulated
in
response
to
oxidative
stress
,
in
the
absence
of
overt
inflammation
.
i-
Proteasomes
are
recruited
to
mitochondria
when
fibroblasts
are
exposed
to
an
excess
of
very
-
long
-chain
fatty
acids
in
response
to
oxidative
stress
.
Antioxidant
treatment
regulates
proteasome
expression
,
prevents
i-proteasome
induction
and
translocation
of
i-proteasomes
to
mitochondria
.
Our
findings
support
a
key
role
of
i-proteasomes
in
quality
control
in
mitochondria
during
oxidative
damage
in
X-
linked
adrenoleukodystrophy
,
and
perhaps
in
other
neurodegenerative
conditions
with
similar
pathogeneses
.
