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Neuroprotective role of lipoic acid against acute toxicity of N-acetylaspartic acid.
[canavan disease]
N-Acetylaspartic
acid
(
NAA
)
accumulates
in
Canavan
disease
,
a
severe
inherited
neurometabolic
disorder
clinically
characterized
by
mental
retardation
,
hypotonia
,
macrocephaly
,
and
seizures
.
The
mechanisms
of
brain
damage
in
this
disease
remain
poorly
understood
.
Recent
studies
developed
by
our
research
group
showed
that
NAA
induces
oxidative
stress
in
vitro
and
in
vivo
in
cerebral
cortex
of
rats
.
Lipoic
acid
is
considered
as
an
efficient
antioxidant
which
can
easily
cross
the
blood
-
brain
barrier
.
Considering
the
absence
of
specific
treatment
to
Canavan
disease
,
this
study
evaluates
the
possible
prevention
of
the
oxidative
stress
promoted
by
NAA
in
vivo
by
the
antioxidant
lipoic
acid
to
preliminarily
evaluate
lipoic
acid
efficacy
against
pro-oxidative
effects
of
NAA
.
Fourteen
-
day
-old
Wistar
rats
received
an
acute
administration
of
0
.
6
mmol
NAA
/
g
body
weight
with
or
without
lipoic
acid
(
40
mg
/
kg
body
weight
)
.
Catalase
(
CAT
)
,
glutathione
peroxidase
(
GPx
)
,
and
glucose
6
-
phosphate
dehydrogenase
activities
,
hydrogen
peroxide
content
,
thiobarbituric
acid-reactive
substances
(
TBA-RS
)
,
spontaneous
chemiluminescence
,
protein
carbonyl
content
,
total
antioxidant
potential
,
and
DNA-protein
cross-links
were
assayed
in
the
cerebral
cortex
of
rats
.
CAT
,
GPx
activities
,
and
total
antioxidant
potential
were
significantly
reduced
,
while
hydrogen
peroxide
content
,
TBA-RS
,
spontaneous
chemiluminescence
,
and
protein
carbonyl
content
were
significantly
enhanced
by
acute
administration
of
NAA
.
Those
effects
were
all
prevented
by
lipoic
acid
pretreatment
.
Our
results
clearly
show
that
lipoic
acid
may
protect
against
the
oxidative
stress
promoted
by
NAA
.
This
could
represent
a
new
therapeutic
approach
to
the
patients
affected
by
Canavan
disease
.
Diseases
Validation
Diseases presenting
"absence of specific treatment"
symptom
canavan disease
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