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Glutathione metabolism enzymes in brain and liver of hyperphenylalaninemic rats and the effect of lipoic acid treatment.
[phenylketonuria]
Phenylketonuria
(
PKU
)
is
a
disorder
caused
by
a
deficiency
in
phenylalanine
hydroxylase
activity
,
which
converts
phenylalanine
(
Phe
)
to
tyrosine
,
leading
to
hyperphenylalaninemia
(
HPA
)
with
accumulation
of
Phe
in
tissues
of
patients
.
The
neuropathophysiology
mechanism
of
disease
remains
unknown
.
However
,
recently
the
involvement
of
oxidative
stress
with
decreased
glutathione
levels
in
PKU
has
been
reported
.
Intracellular
glutathione
(
GSH
)
levels
may
be
maintained
by
the
antioxidant
action
of
lipoic
acid
(
LA
)
.
The
aim
of
this
study
was
to
evaluate
the
activity
of
the
enzymes
involved
in
the
metabolism
and
function
of
GSH
,
such
as
glutathione
peroxidase
(
GSH-Px
)
,
glucose-
6
-
phosphate
dehydrogenase
(
G
6
PD
)
,
glutathione
reductase
(
GR
)
,
glutamate-cysteine
ligase
(
GCL
)
,
glutathione-
S-
transferase
(
GST
)
and
GSH
content
in
brain
and
liver
of
young
rats
subjected
to
a
chemically
induced
model
of
HPA
and
the
effect
of
LA
for
a
week
.
In
brain
,
the
administration
of
Phe
reduced
the
activity
of
the
GSH-Px
,
GR
and
G
6
PD
and
LA
prevented
these
effects
totally
or
partially
.
GCL
activity
was
increased
by
HPA
and
was
not
affect
by
LA
antioxidant
treatment
.
GST
activity
did
not
differ
between
groups
.
GSH
content
was
increased
by
LA
and
decreased
by
HPA
treatment
in
brain
samples
.
Considering
the
liver
,
all
parameters
analyzed
were
increased
in
studied
HPA
animals
and
LA
was
able
to
hinder
some
effects
except
for
the
GCL
,
GST
enzymes
and
GSH
content
.
These
results
suggested
that
HPA
model
alter
the
metabolism
of
GSH
in
rat
brain
and
liver
,
which
may
have
an
important
role
in
the
maintenance
of
GSH
function
in
PKU
although
liver
is
not
a
directly
affected
organ
in
this
disease
.
So
,
an
antioxidant
therapy
with
LA
may
be
useful
in
the
treatment
of
oxidative
stress
in
HPA
.
Diseases
Validation
Diseases presenting
"deficiency in phenylalanine hydroxylase activity"
symptom
phenylketonuria
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