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N-acetylaspartic acid impairs enzymatic antioxidant defenses and enhances hydrogen peroxide concentration in rat brain.
[canavan disease]
N-Acetylaspartic
acid
accumulates
in
Canavan
Disease
,
a
severe
inherited
neurometabolic
disease
clinically
characterized
by
severe
mental
retardation
,
hypotonia
,
macrocephaly
and
generalized
tonic
and
clonic
type
seizures
.
Considering
that
the
mechanisms
of
brain
damage
in
this
disease
remain
poorly
understood
,
in
the
present
study
we
investigated
the
in
vitro
and
in
vivo
effects
of
N-
acetylaspartic
acid
on
the
activities
of
catalase
,
superoxide
dismutase
and
glutathione
peroxidase
,
as
well
as
on
hydrogen
peroxide
concentration
in
cerebral
cortex
of
14
-
day
-old
rats
.
Catalase
and
glutathione
peroxidase
activities
were
significantly
inhibited
,
while
hydrogen
peroxide
concentration
was
significantly
enhanced
by
N-
acetylaspartic
acid
both
in
vitro
and
in
vivo
.
In
contrast
,
superoxide
dismutase
activity
was
not
altered
by
N-
acetylaspartic
acid
.
Our
results
clearly
show
that
N-
acetylaspartic
acid
impairs
the
enzymatic
antioxidant
defenses
in
rat
brain
.
This
could
be
involved
in
the
pathophysiological
mechanisms
responsible
for
the
brain
damage
observed
in
patients
affected
by
Canavan
Disease
.
Diseases
Validation
Diseases presenting
"generalized tonic and clonic type seizures"
symptom
canavan disease
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