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Oxidative stress and adrenocortical insufficiency.
[triple a syndrome]
Maintenance
of
redox
balance
is
essential
for
normal
cellular
functions
.
Any
perturbation
in
this
balance
due
to
increased
reactive
oxygen
species
(
ROS
)
leads
to
oxidative
stress
and
may
lead
to
cell
dysfunction
/
damage
/
death
.
Mitochondria
are
responsible
for
the
majority
of
cellular
ROS
production
secondary
to
electron
leakage
as
a
consequence
of
respiration
.
Furthermore
,
electron
leakage
by
the
cytochrome
P
450
enzymes
may
render
steroidogenic
tissues
acutely
vulnerable
to
redox
imbalance
.
The
adrenal
cortex
,
in
particular
,
is
well
supplied
with
both
enzymatic
(
glutathione
peroxidases
and
peroxiredoxins
)
and
non-enzymatic
(
vitamins
A
,
C
and
E
)
antioxidants
to
cope
with
this
increased
production
of
ROS
due
to
steroidogenesis
.
Nonetheless
oxidative
stress
is
implicated
in
several
potentially
lethal
adrenal
disorders
including
X-
linked
adrenoleukodystrophy
,
triple
A
syndrome
and
most
recently
familial
glucocorticoid
deficiency
.
The
finding
of
mutations
in
antioxidant
defence
genes
in
the
latter
two
conditions
highlights
how
disturbances
in
redox
homeostasis
may
have
an
effect
on
adrenal
steroidogenesis
.
Diseases
Validation
Diseases presenting
"increased production of ros due to steroidogenesis"
symptom
triple a syndrome
x-linked adrenoleukodystrophy
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