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Pivotal role of augmented αB-crystallin in tumor development induced by deficient TSC1/2 complex.
[lymphangioleiomyomatosis]
Tuberous
sclerosis
complex
1
(
TSC
1
)
and
TSC
2
are
suppressors
of
mechanistic
target
of
rapamycin
(
mTOR
)
.
mTOR
is
the
major
component
of
two
protein
complexes
:
mTOR
complex
1
(
mTORC
1
)
and
mTORC
2
.
Inactive
mutation
of
either
TSC
1
or
TSC
2
unleashes
mTOR
signaling
and
consequently
causes
TSC
,
a
benign
tumor
syndrome
affecting
multiple
organs
.
We
report
here
that
expression
of
αB-crystallin
was
upregulated
in
Tsc
1
-
/
-
or
Tsc
2
-
/
-
mouse
embryonic
fibroblasts
,
Eker
rat
uterine
leiomyoma
-derived
Tsc
2
-
deficient
ELT
3
cells
,
mutant
Tsc
2
-
associated
mouse
kidney
tumors
,
and
human
lung
lymphangioleiomyomatosis
nodules
.
αB-crystallin
was
transcriptionally
activated
by
mTOR
complex
2
(
mTORC
2
)
:
nuclear
factor
-kappa
B
(
NFκB
)
signaling
cascade
.
The
augmented
αB-crystallin
was
critical
for
the
migration
,
invasion
and
apoptotic
resistance
of
Tsc
2
-
defective
cells
.
Disruption
of
αB-crystallin
suppressed
Tsc
2
-
null
cell
proliferation
and
tumorigenesis
.
Therefore
,
enhanced
αB-crystallin
has
an
essential
role
in
TSC
1
/
2
complex
deficiency
-mediated
tumorigenesis
,
and
inhibition
of
αB-crystallin
may
complement
the
current
therapy
for
TSC
.
Diseases
Validation
Diseases presenting
"and inhibition of αb-crystallin may complement the current therapy for tsc"
symptom
lymphangioleiomyomatosis
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