Rare Diseases Symptoms Automatic Extraction
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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
.
Diseases
Validation
Diseases presenting
"neurodegeneration"
symptom
adrenomyeloneuropathy
alexander disease
cadasil
canavan disease
classical phenylketonuria
fabry disease
gm1 gangliosidosis
hereditary cerebral hemorrhage with amyloidosis
krabbe disease
neonatal adrenoleukodystrophy
neuralgic amyotrophy
oculocutaneous albinism
pyruvate dehydrogenase deficiency
triple a syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
This symptom has already been validated