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Hepatic damage and oxidative stress induced by Griseofulvin in mice.
[erythropoietic protoporphyria]
Erythropoietic
Protoporphyria
(
EPP
)
is
a
disease
associated
with
ferrochelatase
deficiency
,
which
produces
accumulation
of
protoporphyrin
IX
(
PROTO
IX
)
in
erythrocytes
,
liver
and
skin
.
In
some
cases
,
a
severe
hepatic
failure
and
cholestasis
was
observed
.
Griseofulvin
(
Gris
)
develops
an
experimental
EPP
with
hepatic
manifestations
in
animals
.
The
aim
of
this
work
was
to
further
characterize
this
model
studying
its
effect
on
different
metabolisms
in
mice
Gris
feeding
(
0
-
2
.
5
%
,
7
and
14
days
)
.
PROTO
IX
accumulation
in
liver
,
blood
and
feces
,
induction
of
ALA
-S
activity
,
and
a
low
rate
of
Holo
/
Apo
tryptophan
pyrrolase
activity
was
produced
,
indicating
a
reduction
of
free
heme
pool
.
The
progressive
liver
injury
was
reflected
by
the
aspect
and
the
enlargement
of
liver
and
the
induction
of
hepatic
damage
.
Liver
redox
balance
was
altered
due
to
porphyrin
high
concentrations
;
as
a
consequence
,
the
antioxidant
defense
system
was
disrupted
.
Heme
oxygenase
was
also
induced
,
however
,
at
higher
concentrations
of
antifungal
,
the
free
heme
pool
would
be
so
depleted
that
this
enzyme
would
not
be
necessary
.
In
conclusion
,
our
model
of
Protoporphyria
produced
liver
alterations
similar
to
those
found
in
EPP
patients
.
Diseases
Validation
Diseases presenting
"low rate"
symptom
22q11.2 deletion syndrome
erythropoietic protoporphyria
junctional epidermolysis bullosa
thoracic outlet syndrome
trochlear dysplasia
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