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Evidence of oxidative stress in very long chain fatty acid--treated oligodendrocytes and potentialization of ROS production using RNA interference-directed knockdown of ABCD1 and ACOX1 peroxisomal proteins.
[x-linked adrenoleukodystrophy]
X-
linked
adrenoleukodystrophy
(
X-
ALD
)
and
pseudo
neonatal
adrenoleukodystrophy
(
P-
NALD
)
are
neurodegenerative
demyelinating
diseases
resulting
from
the
functional
loss
of
the
peroxisomal
ATP-binding
cassette
transporter
D
(
ABCD
1
)
and
from
single
peroxisomal
enzyme
deficiency
(
Acyl-
CoA
oxidase
1
:
ACOX
1
)
,
respectively
.
As
these
proteins
are
involved
in
the
catabolism
of
very
long
chain
fatty
acids
(
VLCFA
:
C
2
4
:
0
,
C
2
6
:
0
)
,
X-
ALD
and
P-
NALD
patients
are
characterized
by
the
accumulation
of
VLCFA
in
plasma
and
tissues
.
Since
peroxisomes
are
involved
in
the
metabolism
of
reactive
oxygen
species
(
ROS
)
and
nitrogen
species
(
RNS
)
,
we
examined
the
impact
of
VLCFA
on
the
oxidative
status
of
158
N
murine
oligodendrocytes
expressing
or
not
Abcd
1
or
Acox
1
.
VLCFA
triggers
an
oxidative
stress
characterized
by
an
overproduction
of
ROS
and
RNS
associated
with
lipid
peroxidation
,
protein
carbonylation
,
increased
superoxide
dismutase
(
SOD
)
activity
,
decreased
catalase
activity
and
glutathione
level
.
SiRNA
knockdown
of
Abcd
1
or
Acox
1
increased
ROS
and
RNS
production
even
in
the
absence
of
VLCFA
,
and
especially
potentialized
VLCFA-induced
ROS
overproduction
.
Moreover
,
mainly
in
cells
with
reduced
Acox
1
level
,
the
levels
of
VLCFA
and
neutral
lipids
were
strongly
enhanced
both
in
untreated
and
VLCFA
-
treated
cells
.
Our
data
obtained
on
158
N
murine
oligodendrocytes
highlight
that
VLCFA
induce
an
oxidative
stress
,
and
demonstrate
that
Abcd
1
or
Acox
1
knockdown
contributes
to
disrupt
RedOx
equilibrium
supporting
a
link
between
oxidative
stress
and
the
deficiency
of
Abcd
1
or
Acox
1
peroxisomal
proteins
.
Diseases
Validation
Diseases presenting
"deficiency of abcd1"
symptom
neonatal adrenoleukodystrophy
x-linked adrenoleukodystrophy
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