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The absence of ABCD2 sensitizes mice to disruptions in lipid metabolism by dietary erucic acid.
[x-linked adrenoleukodystrophy]
ABCD
2
(
D
2
)
is
a
peroxisomal
transporter
that
is
highly
abundant
in
adipose
tissue
and
promotes
the
oxidation
of
long
-chain
MUFA
.
Erucic
acid
(
EA
,
22
:
1
ω
9
)
reduces
very
long
chain
saturated
fatty
acids
in
patients
with
X-
linked
adrenoleukodystrophy
but
promotes
dyslipidemia
and
dilated
cardiomyopathy
in
rats
.
To
determine
the
role
of
D
2
in
the
metabolism
of
EA
,
we
challenged
wild-
type
and
D
2
deficient
mice
(
D
2
KO
)
with
an
enriched
EA
diet
.
In
D
2
KO
mice
,
dietary
EA
resulted
in
the
rapid
expansion
of
adipose
tissue
,
adipocyte
hypertrophy
,
hepatic
steatosis
,
and
the
loss
of
glycemic
control
.
However
,
D
2
had
no
impact
on
the
development
of
obesity
phenotypes
in
two
models
of
diet-induced
obesity
.
Although
there
was
a
significant
increase
in
EA
in
liver
of
D
2
KO
mice
,
it
constituted
less
than
2
%
of
all
fatty
acids
.
Metabolites
of
EA
(
20
:
1
,
18
:
1
,
and
16
:
1
)
were
elevated
,
particularly
18
:
1
,
which
accounted
for
50
%
of
all
fatty
acids
.
These
data
indicate
that
the
failure
to
metabolize
EA
in
adipose
results
in
hepatic
metabolism
of
EA
,
disruption
of
the
fatty
acid
profile
,
and
the
development
of
obesity
and
reveal
an
essential
role
for
D
2
in
the
protection
from
dietary
EA
.
Diseases
Validation
Diseases presenting
"obesity"
symptom
acute rheumatic fever
adrenal incidentaloma
aniridia
aromatase deficiency
carcinoma of the gallbladder
cohen syndrome
congenital adrenal hyperplasia
cushing syndrome
cystinuria
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
familial hypocalciuric hypercalcemia
familial mediterranean fever
heparin-induced thrombocytopenia
kabuki syndrome
monosomy 21
phenylketonuria
primary hyperoxaluria type 1
sneddon syndrome
werner syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated