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Lipid oxidation and peroxidation in CNS health and disease: from molecular mechanisms to therapeutic opportunities.
[zellweger syndrome]
Reactive
oxygen
species
(
ROS
)
are
produced
at
low
levels
in
mammalian
cells
by
various
metabolic
processes
,
such
as
oxidative
phosphorylation
by
the
mitochondrial
respiratory
chain
,
NAD
(
P
)
H
oxidases
,
and
arachidonic
acid
oxidative
metabolism
.
To
maintain
physiological
redox
balance
,
cells
have
endogenous
antioxidant
defenses
regulated
at
the
transcriptional
level
by
Nrf
2
/
ARE
.
Oxidative
stress
results
when
ROS
production
exceeds
the
cell
's
ability
to
detoxify
ROS
.
Overproduction
of
ROS
damages
cellular
components
,
including
lipids
,
leading
to
decline
in
physiological
function
and
cell
death
.
Reaction
of
ROS
with
lipids
produces
oxidized
phospholipids
,
which
give
rise
to
4
-
hydroxynonenal
,
4
-
oxo-
2
-
nonenal
,
and
acrolein
.
The
brain
is
susceptible
to
oxidative
damage
due
to
its
high
lipid
content
and
oxygen
consumption
.
Neurodegenerative
diseases
(
AD
,
ALS
,
bipolar
disorder
,
epilepsy
,
Friedreich
's
ataxia
,
HD
,
MS
,
NBIA
,
NPC
,
PD
,
peroxisomal
disorders
,
schizophrenia
,
Wallerian
degeneration
,
Zellweger
syndrome
)
and
CNS
traumas
(
stroke
,
TBI
,
SCI
)
are
problems
of
vast
clinical
importance
.
Free
iron
can
react
with
H
(
2
)
O
(
2
)
via
the
Fenton
reaction
,
a
primary
cause
of
lipid
peroxidation
,
and
may
be
of
particular
importance
for
these
CNS
injuries
and
disorders
.
Cholesterol
is
an
important
regulator
of
lipid
organization
and
the
precursor
for
neurosteroid
biosynthesis
.
Atherosclerosis
,
the
major
risk
factor
for
ischemic
stroke
,
involves
accumulation
of
oxidized
LDL
in
the
arteries
,
leading
to
foam
cell
formation
and
plaque
development
.
This
review
will
discuss
the
role
of
lipid
oxidation
/
peroxidation
in
various
CNS
injuries
/
disorders
.
Diseases
Validation
Diseases presenting
"epilepsy"
symptom
22q11.2 deletion syndrome
adrenomyeloneuropathy
alexander disease
canavan disease
classical phenylketonuria
cohen syndrome
cowden syndrome
familial hypocalciuric hypercalcemia
gm1 gangliosidosis
hereditary cerebral hemorrhage with amyloidosis
hirschsprung disease
homocystinuria without methylmalonic aciduria
kabuki syndrome
locked-in syndrome
lymphangioleiomyomatosis
monosomy 21
neonatal adrenoleukodystrophy
pendred syndrome
phenylketonuria
proteus syndrome
pyruvate dehydrogenase deficiency
sneddon syndrome
wolf-hirschhorn syndrome
zellweger syndrome
This symptom has already been validated