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Oxidative stress is induced in female carriers of X-linked adrenoleukodystrophy.
[adrenomyeloneuropathy]
X-
linked
adrenoleukodystrophy
(
X-
ALD
)
is
a
peroxisomal
disease
biochemically
characterized
by
the
accumulation
of
very
long
chain
fatty
acids
(
VLCFA
)
,
particularly
hexacosanoic
(
C
2
6
:
0
)
and
tetracosanoic
acids
(
C
2
4
:
0
)
in
different
tissues
and
in
biological
fluids
and
clinically
characterized
by
central
and
peripheral
demyelination
and
adrenal
insufficiency
.
A
considerable
number
of
heterozygotes
(
HTZ
)
for
X-
ALD
develop
neurological
symptoms
like
spinal
cord
involvement
resembling
milder
forms
of
adrenomyeloneuropathy
.
However
,
the
mechanisms
of
brain
damage
in
hemizygotes
and
heterozygotes
X-
ALD
individuals
are
poorly
understood
.
Considering
that
oxidative
stress
was
involved
in
various
neurodegenerative
disorders
and
that
in
a
previous
study
we
showed
evidence
that
oxidative
stress
is
probably
involved
in
the
pathophysiology
of
X-
ALD
symptomatic
patients
,
in
the
present
study
we
evaluated
various
oxidative
stress
parameters
,
namely
thiobarbituric
acid-reactive
substances
(
TBA-RS
)
,
total
antioxidant
status
(
TAS
)
and
total
antioxidant
reactivity
(
TAR
)
in
plasma
of
HTZ
individuals
for
X-
ALD
.
It
was
observed
that
female
carriers
present
a
significant
increase
of
TBA-RS
measurement
,
indicating
a
stimulation
of
lipid
peroxidation
,
as
well
as
a
decrease
of
TAR
,
reflecting
a
deficient
capacity
to
rapidly
handle
an
increase
of
reactive
species
.
These
results
indicate
that
oxidative
stress
is
involved
in
the
pathophysiology
of
heterozygotes
for
X-
ALD
.
