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Caffeic acid phenethyl ester induces adrenoleukodystrophy (Abcd2) gene in human X-ALD fibroblasts and inhibits the proinflammatory response in Abcd1/2 silenced mouse primary astrocytes.
[x-linked adrenoleukodystrophy]
X-
linked
adrenoleukodystrophy
(
X-
ALD
)
is
a
peroxisomal
disorder
caused
by
mutations
in
the
ABCD
1
gene
.
Accumulation
of
very
long
chain
fatty
acids
(
VLCFA
)
that
have
been
attributed
to
reduced
peroxisomal
VLCFA
β-oxidation
activity
are
the
hallmark
of
the
disease
.
Overexpression
of
ABCD
2
gene
,
the
closest
homolog
of
ABCD
1
,
has
been
shown
to
compensate
for
ABCD
1
,
thus
correcting
the
VLCFA
derangement
.
The
accumulation
of
VLCFA
leads
to
a
neuroinflammatory
disease
process
associated
with
demyelination
of
the
cerebral
white
matter
.
The
present
study
underlines
the
importance
of
caffeic
acid
phenethyl
ester
(
CAPE
)
in
inducing
the
expression
of
ABCD
2
(
ALDRP
)
,
and
normalizing
the
peroxisomal
β-oxidation
as
well
as
the
levels
of
saturated
and
monounsaturated
VLCFAs
in
cultured
human
skin
fibroblasts
of
X-
ALD
patients
.
The
expression
of
ELOVL
1
,
the
single
elongase
catalyzing
the
synthesis
of
both
saturated
VLCFA
(
C
2
6
:
0
)
and
mono-unsaturated
VLCFA
(
C
2
6
:
1
)
,
was
also
reduced
by
CAPE
treatment
.
Importantly
,
CAPE
upregulated
Abcd
2
expression
and
peroxisomal
β-oxidation
and
lowered
the
VLCFA
levels
in
Abcd
1
-
deficient
U
87
astrocytes
and
B
12
oligodendrocytes
.
In
addition
,
using
Abcd
1
/
Abcd
2
-
silenced
mouse
primary
astrocytes
we
examined
the
effects
of
CAPE
in
VLCFA-induced
inflammatory
response
.
CAPE
treatment
decreased
the
inflammatory
response
as
the
expression
of
inducible
nitric
oxide
synthase
,
inflammatory
cytokine
,
and
activation
of
NF-κB
in
Abcd
1
/
Abcd
2
-
silenced
mouse
primary
astrocytes
was
reduced
.
The
observations
indicate
that
CAPE
corrects
both
the
metabolic
disease
of
VLCFA
as
well
as
secondary
inflammatory
disease
;
therefore
,
it
may
be
a
potential
drug
candidate
to
be
tested
for
X-
ALD
therapy
in
humans
.
Diseases
Validation
Diseases presenting
"skin fibroblasts"
symptom
child syndrome
cowden syndrome
cystinuria
dentinogenesis imperfecta
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
gm1 gangliosidosis
homocystinuria without methylmalonic aciduria
krabbe disease
malignant atrophic papulosis
monosomy 21
neonatal adrenoleukodystrophy
pyruvate dehydrogenase deficiency
werner syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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