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Mitochondrial dysfunction in demyelinating diseases.
[x-linked adrenoleukodystrophy]
Dysfunction
of
the
mitochondrial
(
mt
)
system
is
thought
to
play
an
important
role
in
the
mechanism
of
progression
of
various
neurodegenerative
disorders
,
including
demyelinating
disorders
.
They
are
characterized
by
neuroinflammation
,
ultimately
leading
to
neurodegeneration
.
Mitochondria
(
mt
)
dysfunction
is
closely
related
to
the
mechanism
of
neuroinflammation
,
causing
increased
production
of
reactive
oxygen
species
,
which
is
detrimental
to
neurons
and
glia
.
Vice
versa
,
neuroinflammation
is
increasingly
recognized
to
produce
mt
failure
,
which
then
contributes
to
further
neuronal
injury
and
degeneration
.
Multiple
sclerosis
and
X-
linked
adrenoleukodystrophy
are
examples
of
neurodemyelinating
diseases
that
despite
having
a
diverse
etiology
have
in
common
a
progressive
course
and
significant
neuroinflammation
and
neurodegeneration
,
leading
to
severe
neurologic
disability
.
The
scientific
community
has
become
increasingly
interested
in
how
mt
dysfunction
relates
to
neuroinflammation
and
demyelination
and
what
role
it
may
play
in
the
natural
history
of
progressive
demyelinating
diseases
.
Research
studies
investigating
how
mt
failure
contributes
to
the
progression
of
these
conditions
are
emerging
.
A
better
understanding
of
the
role
of
oxidative
stress
in
progressive
inflammatory
demyelinating
diseases
might
generate
new
potential
neuroprotective
therapeutic
approaches
for
these
devastating
neurologic
conditions
.