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The oxidative molecular regulation mechanism of NOX in children with phenylketonuria.
[phenylketonuria]
Phenylketonuria
(
PKU
)
is
the
most
frequent
inherited
disorder
of
amino
acid
metabolism
.
In
our
previous
work
,
we
investigated
the
role
of
NADPH
oxidase
(
NOX
)
in
a
Pahenu
2
-
BTBR
PKU
mouse
model
,
and
an
in
vitro
cell
culture
model
of
PKU
.
In
the
current
study
,
we
evaluated
various
oxidative
stress
parameters
,
namely
total
antioxidant
capacity
(
T
-AOC
)
,
glutathione
(
GSH
)
and
maleic
dialdehyde
(
MDA
)
in
the
plasma
of
40
PKU
children
,
for
further
investigating
the
oxidative
molecular
regulation
mechanism
of
NOX
in
PKU
.
It
was
observed
that
T
-AOC
and
GSH
markedly
decreased
in
PKU
as
compared
with
the
control
group
(
P
<
0
.
01
)
,
and
seemed
to
correlate
negatively
with
Phe
level
.
However
,
there
was
no
statistical
difference
in
MDA
level
among
the
three
groups
.
And
8
-
isoprostane
in
the
blood
samples
of
PKU
2
groups
was
slightly
higher
than
control
group
(
P
<
0
.
05
)
.
Additionally
,
mRNA
levels
of
subunits
of
NOX
included
p
47
(
phox
)
and
p
67
(
phox
)
significantly
increased
in
PKU
group
(
P
<
0
.
01
)
.
These
results
reflected
that
NOX
is
the
important
source
of
reactive
oxygen
species
and
is
involved
in
the
oxidative
molecular
regulation
mechanism
in
PKU
,
which
shows
a
new
perspective
toward
understanding
the
biological
underpinnings
of
PKU
.
Diseases
Validation
Diseases presenting
"oxidative molecular regulation mechanism"
symptom
phenylketonuria
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