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N-acetylaspartic acid promotes oxidative stress in cerebral cortex of rats.
[canavan disease]
N-
acetylaspartic
acid
accumulates
in
Canavan
Disease
,
a
severe
leukodystrophy
characterized
by
swelling
and
spongy
degeneration
of
the
white
matter
of
the
brain
.
This
inherited
metabolic
disease
,
caused
by
deficiency
of
the
enzyme
aspartoacylase
,
is
clinically
characterized
by
severe
mental
retardation
,
hypotonia
and
macrocephaly
,
and
also
generalized
tonic
and
clonic
type
seizures
in
about
half
of
the
patients
.
Considering
that
the
mechanisms
of
brain
damage
in
this
disease
remain
not
fully
understood
,
in
the
present
study
we
investigated
whether
oxidative
stress
is
elicited
by
N-
acetylaspartic
acid
.
The
in
vitro
effect
of
N-
acetylaspartic
acid
(
10
-
80
mM
)
was
studied
on
oxidative
stress
parameters
:
total
radical-trapping
antioxidant
potential
(
TRAP
)
,
total
antioxidant
reactivity
(
TAR
)
,
chemiluminescence
,
thiobarbituric
acid-reactive
substances
(
TBA-RS
)
,
reduced
glutathione
content
,
sufhydryl
content
and
carbonyl
content
in
the
cerebral
cortex
of
14
-
day
-old
rats
.
The
effect
of
the
acute
administration
of
N-
acetylaspartic
acid
(
0
.
1
-
0
.
6
mmol
/
g
body
weight
)
was
studied
on
TRAP
,
TAR
,
carbonyl
content
,
chemiluminescence
and
TBA-RS
.
TRAP
,
TAR
,
reduced
glutathione
content
and
sulfhydryl
content
were
significantly
reduced
,
while
chemiluminescence
,
TBA-RS
and
carbonyl
content
were
significantly
enhanced
by
N-
acetylaspartic
acid
in
vitro
.
The
enhancement
in
TBA-RS
promoted
by
N-
acetylaspartic
acid
was
completely
prevented
by
ascorbic
acid
plus
Trolox
,
and
partially
prevented
by
glutathione
and
dithiothreitol
.
The
acute
administration
of
N-
acetylaspartic
acid
also
significantly
reduced
TRAP
and
TAR
,
and
significantly
enhanced
carbonyl
content
,
chemiluminescence
and
TBA-RS
.
Our
results
indicate
that
N-
acetylaspartic
acid
promotes
oxidative
stress
by
stimulating
lipid
peroxidation
,
protein
oxidation
and
by
decreasing
non-enzymatic
antioxidant
defenses
in
rat
brain
.
This
could
be
another
pathophysiological
mechanism
involved
in
Canavan
Disease
.
Diseases
Validation
Diseases presenting
"is clinically characterized by severe mental retardation"
symptom
canavan disease
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