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alpha-Synuclein abnormalities in mouse models of peroxisome biogenesis disorders.
[zellweger syndrome]
alpha-
Synuclein
(
alphaS
)
is
a
presynaptic
protein
implicated
in
Parkinson
's
disease
(
PD
)
.
Growing
evidence
implicates
mitochondrial
dysfunction
,
oxidative
stress
,
and
alphaS-lipid
interactions
in
the
gradual
accumulation
of
alphaS
in
pathogenic
forms
and
its
deposition
in
Lewy
bodies
,
the
pathological
hallmark
of
PD
and
related
synucleinopathies
.
The
peroxisomal
biogenesis
disorders
(
PBD
)
,
with
Zellweger
syndrome
serving
as
the
prototype
of
this
group
,
are
characterized
by
malformed
and
functionally
impaired
peroxisomes
.
Here
we
utilized
the
PBD
mouse
models
Pex
2
-
/
-
,
Pex
5
-
/
-
,
and
Pex
13
-
/
-
to
study
the
potential
effects
of
peroxisomal
dysfunction
on
alphaS-related
pathogenesis
.
We
found
increased
alphaS
oligomerization
and
phosphorylation
and
its
increased
deposition
in
cytoplasmic
inclusions
in
these
PBD
mouse
models
.
Furthermore
,
we
show
that
alphaS
abnormalities
correlate
with
the
altered
lipid
metabolism
and
,
specifically
,
with
accumulation
of
long
chain
,
n-
6
polyunsaturated
fatty
acids
that
occurs
in
the
PBD
models
.
Diseases
Validation
Diseases presenting
"altered lipid metabolism"
symptom
zellweger syndrome
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