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Amplification of FRS2 and activation of FGFR/FRS2 signaling pathway in high-grade liposarcoma.
[dedifferentiated liposarcoma]
Fibroblast
growth
factor
(
FGF
)
receptor
(
FGFR
)
substrate
2
(
FRS
2
)
is
an
adaptor
protein
that
plays
a
critical
role
in
FGFR
signaling
.
FRS
2
is
located
on
chromosome
12
q
13
-
15
that
is
frequently
amplified
in
liposarcomas
.
The
significance
of
FRS
2
and
FGFR
signaling
in
high
-grade
liposarcomas
is
unknown
.
Herein
,
we
first
comparatively
examined
the
amplification
and
expression
of
FRS
2
with
CDK
4
and
MDM
2
in
dedifferentiated
liposarcoma
(
DDLS
)
and
undifferentiated
high
-grade
pleomorphic
sarcoma
(
UHGPS
)
.
Amplification
and
expression
of
the
three
genes
were
identified
in
90
%
to
100
%
(
9
-
11
of
11
)
of
DDLS
,
whereas
that
of
FRS
2
,
CDK
4
,
and
MDM
2
were
observed
in
55
%
(
41
of
75
)
,
48
%
(
36
of
75
)
,
and
44
%
(
33
/
75
)
of
clinically
diagnosed
UHGPS
,
suggesting
that
these
"
UHGPS
"
may
represent
DDLS
despite
lacking
histologic
evidence
of
lipoblasts
.
Immunohistochemical
analysis
of
phosphorylated
FRS
2
protein
indicated
that
the
FGFR
/
FRS
2
signaling
axis
was
generally
activated
in
about
75
%
of
FRS
2
-
positive
high
-grade
liposarcomas
.
Moreover
,
we
found
that
FRS
2
and
FGFRs
proteins
are
highly
expressed
and
functional
in
three
high
-grade
liposarcoma
cell
lines
:
FU-
DDLS
-
1
,
LiSa-
2
,
and
SW
872
.
Importantly
,
the
FGFR
selective
inhibitor
NVP-BGJ-
398
significantly
inhibited
the
growth
of
FU-
DDLS
-
1
and
LiSa-
2
cells
with
a
concomitant
suppression
of
FGFR
signal
transduction
.
Attenuation
of
FRS
2
protein
in
FU-
DDLS
-
1
and
LiSa-
2
cell
lines
decreased
the
phosphorylated
extracellular
signal-regulated
kinase
1
/
2
and
AKT
and
repressed
cell
proliferation
.
These
findings
indicate
that
analysis
of
FRS
2
in
combination
with
CDK
4
and
MDM
2
will
more
accurately
characterize
pathologic
features
of
high
-grade
liposarcomas
.
Activated
FGFR
/
FRS
2
signaling
may
play
a
functional
role
in
the
development
of
high
-grade
liposarcomas
,
therefore
,
serve
as
a
potential
therapeutic
target
.
Diseases
Validation
Diseases presenting
"growth factor"
symptom
22q11.2 deletion syndrome
achondroplasia
adrenal incidentaloma
aniridia
cadasil
cholangiocarcinoma
coats disease
dedifferentiated liposarcoma
dentin dysplasia
dentinogenesis imperfecta
dystrophic epidermolysis bullosa
esophageal carcinoma
esophageal squamous cell carcinoma
hodgkin lymphoma, classical
holt-oram syndrome
inclusion body myositis
kallmann syndrome
krabbe disease
liposarcoma
lymphangioleiomyomatosis
oculocutaneous albinism
oral submucous fibrosis
pleomorphic liposarcoma
primary effusion lymphoma
primary hyperoxaluria type 1
severe combined immunodeficiency
systemic capillary leak syndrome
von hippel-lindau disease
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
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