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Disruption of oxidative phosphorylation and synaptic Na(+), K(+)-ATPase activity by pristanic acid in cerebellum of young rats.
[zellweger syndrome]
Peroxisomal
biogenesis
disorders
(
PBD
)
are
inherited
disorders
clinically
manifested
by
neurological
symptoms
and
brain
abnormalities
,
in
which
the
cerebellum
is
usually
involved
.
Biochemically
,
patients
affected
by
these
neurodegenerative
diseases
accumulate
branched-chain
fatty
acids
,
including
pristanic
acid
(
Prist
)
in
the
brain
and
other
tissues
.
In
the
present
investigation
we
studied
the
in
vitro
influence
of
Prist
,
at
doses
found
in
PBD
,
on
oxidative
phosphorylation
,
by
measuring
the
activities
of
the
respiratory
chain
complexes
I
-IV
and
ATP
production
,
as
well
as
on
creatine
kinase
and
synaptic
Na
(
+
)
,
K
(
+
)
-
ATPase
activities
in
rat
cerebellum
.
Prist
significantly
decreased
complexes
I
-
III
(
65
%
)
,
II
(
40
%
)
and
especially
II
-
III
(
90
%
)
activities
,
without
altering
the
activities
of
complex
IV
of
the
respiratory
chain
and
creatine
kinase
.
Furthermore
,
ATP
formation
and
synaptic
Na
(
+
)
,
K
(
+
)
-
ATPase
activity
were
markedly
inhibited
(
80
-
90
%
)
by
Prist
.
We
also
observed
that
this
fatty
acid
altered
mitochondrial
and
synaptic
membrane
fluidity
that
may
have
contributed
to
its
inhibitory
effects
on
the
activities
of
the
respiratory
chain
complexes
and
Na
(
+
)
,
K
(
+
)
-
ATPase
.
Considering
the
importance
of
oxidative
phosphorylation
for
mitochondrial
homeostasis
and
of
Na
(
+
)
,
K
(
+
)
-
ATPase
for
the
maintenance
of
cell
membrane
potential
,
the
present
data
indicate
that
Prist
compromises
brain
bioenergetics
and
neurotransmission
in
cerebellum
.
We
postulate
that
these
pathomechanisms
may
contribute
to
the
cerebellar
alterations
observed
in
patients
affected
by
PBD
in
which
Prist
is
accumulated
.
Diseases
Validation
Diseases presenting
"cerebellar alterations"
symptom
zellweger syndrome
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