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The effect of bone marrow transplantation on oxidative stress in X-linked adrenoleukodystrophy.
[x-linked adrenoleukodystrophy]
Oxidative
stress
plays
an
important
role
in
the
pathophysiology
of
neurodegenerative
diseases
,
including
X-
linked
adrenoleukodystrophy
(
X-
ALD
)
.
In
the
present
work
,
we
evaluated
lipid
(
malondialdehyde
[
MDA
]
content
)
and
protein
(
sulfhydryl
and
carbonyl
contents
)
oxidative
damage
parameters
in
plasma
from
X-
ALD
patients
before
and
after
bone
marrow
transplant
(
BMT
)
,
in
order
to
verify
if
this
treatment
is
capable
to
alter
the
oxidative
parameters
studied
.
We
also
evaluated
the
plasma
concentration
of
hexacosanoic
acid
(
C
2
6
:
0
)
from
X-
ALD
patients
and
correlated
it
with
the
oxidative
damage
parameters
investigated
.
We
observed
that
MDA
content
was
significantly
increased
in
plasma
of
X-
ALD
patients
before
BMT
and
after
BMT
when
compared
to
controls
,
and
that
it
was
significantly
reduced
in
plasma
of
X-
ALD
after
BMT
when
compared
to
the
before
BMT
group
.
These
results
indicate
that
lipid
peroxidation
is
stimulated
in
X-
ALD
patients
but
there
is
a
significant
reduction
of
lipid
peroxidation
after
BMT
.
Next
,
we
observed
a
significant
reduction
of
sulfhydryl
content
in
plasma
of
X-
ALD
patients
before
BMT
compared
to
controls
indicating
protein
oxidative
damage
and
that
this
measurement
was
increased
in
these
patients
after
BMT
as
compared
to
before
BMT
.
We
found
no
significant
differences
in
plasma
carbonyl
content
in
X-
ALD
patients
before
and
after
BMT
as
compared
to
controls
.
However
,
we
observed
a
significant
reduction
in
this
parameter
in
X-
ALD
patients
after
BMT
compared
to
before
BMT
.
Finally
,
C
2
6
:
0
plasma
concentration
was
significantly
reduced
in
X-
ALD
patients
after
BMT
when
compared
to
before
BMT
.
We
found
no
significant
correlations
between
MDA
and
carbonyl
values
with
C
2
6
:
0
levels
of
the
patients
before
BMT
and
after
BMT
,
but
a
significant
inverse
correlation
between
sulfhydryl
content
and
C
2
6
:
0
levels
was
detected
.
In
conclusion
,
the
present
study
reinforces
the
hypothesis
that
lipid
peroxidation
and
protein
damage
are
induced
in
plasma
of
X-
ALD
patients
and
,
in
addition
,
demonstrates
that
BMT
treatment
is
capable
to
reduce
this
pathogenic
process
.
Taken
together
,
the
data
obtained
from
plasma
of
X-
ALD
patients
before
and
after
BMT
showing
induction
and
protection
,
respectively
,
of
oxidative
stress
,
allowed
to
suggest
that
BMT
,
when
well
succeeded
and
under
the
recommendations
,
is
effective
to
reduce
C
2
6
:
0
plasma
levels
and
the
increased
lipid
and
protein
oxidative
damage
in
X-
ALD
.
Diseases
Validation
Diseases presenting
"reduction of lipid peroxidation"
symptom
x-linked adrenoleukodystrophy
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