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Lipid rafts are essential for peroxisome biogenesis in HepG2 cells.
[zellweger syndrome]
Peroxisomes
are
particularly
abundant
in
the
liver
and
are
involved
in
bile
salt
synthesis
and
fatty
acid
metabolism
.
Peroxisomal
membrane
proteins
(
PMPs
)
are
required
for
peroxisome
biogenesis
[
e
.
g
.
,
the
interacting
peroxisomal
biogenesis
factors
Pex
13
p
and
Pex
14
p
]
and
its
metabolic
function
[
e
.
g
.
,
the
adenosine
triphosphate-binding
cassette
transporters
adrenoleukodystrophy
protein
(
ALDP
)
and
PMP
70
]
.
Impaired
function
of
PMPs
is
the
underlying
cause
of
Zellweger
syndrome
and
X-
linked
adrenoleukodystrophy
.
Here
we
studied
for
the
first
time
the
putative
association
of
PMPs
with
cholesterol-enriched
lipid
rafts
and
their
function
in
peroxisome
biogenesis
.
Lipid
rafts
were
isolated
from
Triton
X-
100
-
lysed
or
Lubrol
WX-lysed
HepG
2
cells
and
analyzed
for
the
presence
of
various
PMPs
by
western
blotting
.
Lovastatin
and
methyl-
beta
-cyclodextrin
were
used
to
deplete
cholesterol
and
disrupt
lipid
rafts
in
HepG
2
cells
,
and
this
was
followed
by
immunofluorescence
microscopy
to
determine
the
subcellular
location
of
catalase
and
PMPs
.
Cycloheximide
was
used
to
inhibit
protein
synthesis
.
Green
fluorescent
protein-tagged
fragments
of
PMP
70
and
ALDP
were
analyzed
for
their
lipid
raft
association
.
PMP
70
and
Pex
14
p
were
associated
with
Triton
X-
100
-
resistant
rafts
,
ALDP
was
associated
with
Lubrol
WX-resistant
rafts
,
and
Pex
13
p
was
not
lipid
raft-associated
in
HepG
2
cells
.
The
minimal
peroxisomal
targeting
signals
in
ALDP
and
PMP
70
were
not
sufficient
for
lipid
raft
association
.
Cholesterol
depletion
led
to
dissociation
of
PMPs
from
lipid
rafts
and
impaired
sorting
of
newly
synthesized
catalase
and
ALDP
but
not
Pex
14
p
and
PMP
70
.
Repletion
of
cholesterol
to
these
cells
efficiently
reestablished
the
peroxisomal
sorting
of
catalase
but
not
ALDP
.
Human
PMPs
are
differentially
associated
with
lipid
rafts
independently
of
the
protein
homology
and
/
or
their
functional
interaction
.
Cholesterol
is
required
for
peroxisomal
lipid
raft
assembly
and
peroxisome
biogenesis
.
Diseases
Validation
Diseases presenting
"first time"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
adrenomyeloneuropathy
alpha-thalassemia
aniridia
aromatase deficiency
canavan disease
carcinoma of the gallbladder
cholangiocarcinoma
classical phenylketonuria
congenital adrenal hyperplasia
congenital toxoplasmosis
cowden syndrome
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dentin dysplasia
dentinogenesis imperfecta
dracunculiasis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
fabry disease
familial mediterranean fever
gm1 gangliosidosis
harlequin ichthyosis
heparin-induced thrombocytopenia
hirschsprung disease
hodgkin lymphoma, classical
holt-oram syndrome
hydrocephalus with stenosis of the aqueduct of sylvius
junctional epidermolysis bullosa
kabuki syndrome
kallmann syndrome
liposarcoma
locked-in syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
monosomy 21
neuralgic amyotrophy
oculocutaneous albinism
oligodontia
omenn syndrome
oral submucous fibrosis
papillon-lefèvre syndrome
pendred syndrome
phenylketonuria
primary effusion lymphoma
primary hyperoxaluria type 1
severe combined immunodeficiency
sneddon syndrome
triple a syndrome
trochlear dysplasia
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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