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Pristanic Acid provokes lipid, protein, and DNA oxidative damage and reduces the antioxidant defenses in cerebellum of young rats.
[zellweger syndrome]
Zellweger
syndrome
(
ZS
)
and
some
peroxisomal
diseases
are
severe
inherited
disorders
mainly
characterized
by
neurological
symptoms
and
cerebellum
abnormalities
,
whose
pathogenesis
is
poorly
understood
.
Biochemically
,
these
diseases
are
mainly
characterized
by
accumulation
of
pristanic
acid
(
Prist
)
and
other
fatty
acids
in
the
brain
and
other
tissues
.
In
this
work
,
we
evaluated
the
in
vitro
influence
of
Prist
on
redox
homeostasis
by
measuring
lipid
,
protein
,
and
DNA
damage
,
as
well
as
the
antioxidant
defenses
and
the
activities
of
aconitase
and
α-ketoglutarate
dehydrogenase
in
cerebellum
of
30
-
day
-old
rats
.
The
effect
of
Prist
on
DNA
damage
was
also
evaluated
in
blood
of
these
animals
.
Some
parameters
were
also
evaluated
in
cerebellum
from
neonatal
rats
and
in
cerebellum
neuronal
cultures
.
Prist
significantly
increased
malondialdehyde
(
MDA
)
levels
and
carbonyl
formation
and
reduced
sulfhydryl
content
and
glutathione
(
GSH
)
concentrations
in
cerebellum
of
young
rats
.
It
also
caused
DNA
strand
damage
in
cerebellum
and
induced
a
high
micronuclei
frequency
in
blood
.
On
the
other
hand
,
this
fatty
acid
significantly
reduced
α-ketoglutarate
dehydrogenase
and
aconitase
activities
in
rat
cerebellum
.
We
also
verified
that
Prist-induced
increase
of
MDA
levels
was
totally
prevented
by
melatonin
and
attenuated
by
α-tocopherol
but
not
by
the
nitric
oxide
synthase
inhibitor
N
(
ω
)
-
nitro-
L-
arginine
methyl
ester
,
indicating
the
involvement
of
reactive
oxygen
species
in
this
effect
.
Cerebellum
from
neonate
rats
also
showed
marked
alterations
of
redox
homeostasis
,
including
an
increase
of
MDA
levels
and
a
decrease
of
sulfhydryl
content
and
GSH
concentrations
elicited
by
Prist
.
Finally
,
Prist
provoked
an
increase
of
dichlorofluorescein
(
DCFH
)
oxidation
in
cerebellum-cultivated
neurons
.
Our
present
data
indicate
that
Prist
compromises
redox
homeostasis
in
rat
cerebellum
and
blood
and
inhibits
critical
enzymes
of
the
citric
acid
cycle
that
are
susceptible
to
free
radical
attack
.
The
present
findings
may
contribute
to
clarify
the
pathogenesis
of
the
cerebellar
alterations
observed
in
patients
affected
by
ZS
and
some
peroxisomal
disorders
in
which
Prist
is
accumulated
.
Diseases
Validation
Diseases presenting
"in cerebellum neuronal cultures"
symptom
zellweger syndrome
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