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Pipecolic acid induces oxidative stress in vitro in cerebral cortex of young rats and the protective role of lipoic acid.
[zellweger syndrome]
Pipecolic
acid
(
PA
)
levels
are
increased
in
severe
metabolic
disorders
of
the
central
nervous
system
such
as
Zellweger
syndrome
,
infantile
Refsum
disease
,
neonatal
adrenoleukodystrophy
and
hyperlysinemia
.
The
affected
individuals
present
progressive
neurological
dysfunction
,
hypotonia
and
growth
retardation
.
The
mechanisms
of
brain
damage
of
these
disorders
remain
poorly
understood
.
Since
PA
catabolism
can
produce
H
2
O
2
by
oxidases
,
oxidative
stress
may
be
a
possible
mechanism
involved
in
the
pathophysiology
of
these
diseases
.
Lipoic
acid
(
LA
)
is
considered
an
efficient
antioxidant
and
has
been
shown
to
prevent
oxidative
stress
in
experimental
models
of
many
disorders
of
the
neurologic
system
.
Considering
that
to
our
knowledge
no
study
investigated
the
role
of
PA
on
oxidative
stress
,
in
the
present
work
we
investigated
the
in
vitro
effects
of
PA
on
some
oxidative
stress
parameters
and
evaluated
the
LA
efficacy
against
possible
pro-oxidant
effects
of
PA
in
cerebral
cortex
of
14
-
day
-old
rats
.
The
activities
of
catalase
(
CAT
)
,
glutathione
peroxidase
(
GPx
)
,
glucose
6
-
phosphate
dehydrogenase
(
G
6
PD
)
,
and
glutathione
S-
transferase
(
GST
)
along
with
reduced
glutathione
(
GSH
)
content
were
significantly
decreased
,
while
superoxide
dismutase
(
SOD
)
activity
and
thiobarbituric
acid-reactive
substances
(
TBA-RS
)
were
significantly
enhanced
by
PA
.
LA
was
able
to
prevent
these
effects
by
improving
the
activity
of
antioxidant
enzymes
,
increasing
GSH
content
and
reducing
TBA-RS
.
In
contrast
,
glutathione
reductase
and
6
-
phosphogluconate
dehydrogenase
activities
and
sulfhydryl
content
were
not
affected
.
Taken
together
,
it
may
be
presumed
that
PA
in
vitro
elicits
oxidative
stress
and
LA
is
able
to
prevent
these
effects
.
Diseases
Validation
Diseases presenting
"thiobarbituric acid-reactive substances"
symptom
adrenomyeloneuropathy
canavan disease
zellweger syndrome
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