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SRC kinase is a novel therapeutic target in lymphangioleiomyomatosis.
[lymphangioleiomyomatosis]
Lymphangioleiomyomatosis
(
LAM
)
is
a
progressive
cystic
lung
disease
affecting
some
women
with
tuberous
sclerosis
complex
(
TSC
)
.
Sporadic
LAM
can
develop
in
women
without
TSC
,
owing
to
somatic
mutations
in
the
TSC
2
gene
.
Accumulating
evidence
supports
the
view
of
LAM
as
a
low
-grade
,
destructive
,
metastasizing
neoplasm
.
The
mechanisms
underlying
the
metastatic
capability
of
LAM
cells
remain
poorly
understood
.
The
observed
behavior
of
LAM
cells
with
respect
to
their
infiltrative
growth
pattern
,
metastatic
potential
,
and
altered
cell
differentiation
bears
similarity
to
cells
undergoing
epithelial-mesenchymal
transition
.
Here
,
we
report
increased
levels
of
active
Src
kinase
in
LAM
lungs
and
in
TSC
2
(
-
/
-
)
cells
,
caused
by
a
reduction
of
autophagy
.
Furthermore
,
increased
Src
kinase
activation
promoted
migration
,
invasion
,
and
inhibition
of
E
-
cadherin
expression
in
TSC
2
(
-
/
-
)
cells
by
upregulating
the
transcription
factor
Snail
.
Notably
,
Src
kinase
inhibitors
reduced
migration
and
invasion
properties
of
TSC
2
(
-
/
-
)
cells
and
attenuated
lung
colonization
of
intravenously
injected
TSC
2
(
-
/
-
)
cells
in
vivo
to
a
greater
extent
than
control
TSC
2
(
+
/
+
)
cells
.
Our
results
reveal
mechanistic
basis
for
the
pathogenicity
of
LAM
cells
and
they
rationalize
Src
kinase
as
a
novel
therapeutic
target
for
treatment
of
LAM
and
TSC
.
Diseases
Validation
Diseases presenting
"lung colonization"
symptom
lymphangioleiomyomatosis
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