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Intracerebroventricular administration of N-acetylaspartic acid impairs antioxidant defenses and promotes protein oxidation in cerebral cortex of rats.
[canavan disease]
N-
acetylaspartic
acid
(
NAA
)
is
the
biochemical
hallmark
of
Canavan
Disease
,
an
inherited
metabolic
disease
caused
by
deficiency
of
aspartoacylase
activity
.
NAA
is
an
immediate
precursor
for
the
enzyme-mediated
biosynthesis
of
N-
acetylaspartylglutamic
acid
(
NAAG
)
,
whose
concentration
is
also
increased
in
urine
and
cerebrospinal
fluid
of
patients
affected
by
CD
.
This
neurodegenerative
disorder
is
clinically
characterized
by
severe
mental
retardation
,
hypotonia
and
macrocephaly
,
and
generalized
tonic
and
clonic
type
seizures
.
Considering
that
the
mechanisms
of
brain
damage
in
this
disease
remain
not
fully
understood
,
in
the
present
study
we
investigated
whether
intracerebroventricular
administration
of
NAA
or
NAAG
elicits
oxidative
stress
in
cerebral
cortex
of
30
-
day
-old
rats
.
NAA
significantly
reduced
total
radical-trapping
antioxidant
potential
,
catalase
and
glucose
6
-
phosphate
dehydrogenase
activities
,
whereas
protein
carbonyl
content
and
superoxide
dismutase
activity
were
significantly
enhanced
.
Lipid
peroxidation
indices
and
glutathione
peroxidase
activity
were
not
affected
by
NAA
.
In
contrast
,
NAAG
did
not
alter
any
of
the
oxidative
stress
parameters
tested
.
Our
results
indicate
that
intracerebroventricular
administration
of
NAA
impairs
antioxidant
defenses
and
induces
oxidative
damage
to
proteins
,
which
could
be
involved
in
the
neurotoxicity
of
NAA
accumulation
in
CD
patients
.
Diseases
Validation
Diseases presenting
"deficiency of aspartoacylase activity"
symptom
canavan disease
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