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Laminin-332 coordinates mechanotransduction and growth cone bifurcation in sensory neurons.
[junctional epidermolysis bullosa]
Laminin-
332
is
a
major
component
of
the
dermo-epidermal
skin
basement
membrane
and
maintains
skin
integrity
.
The
transduction
of
mechanical
force
into
electrical
signals
by
sensory
endings
in
the
skin
requires
mechanosensitive
channels
.
We
found
that
mouse
epidermal
keratinocytes
produce
a
matrix
that
is
inhibitory
for
sensory
mechanotransduction
and
that
the
active
molecular
component
is
laminin-
332
.
Substrate-bound
laminin-
332
specifically
suppressed
one
type
of
mechanosensitive
current
(
rapidly
adapting
)
independently
of
integrin-receptor
activation
.
This
mechanotransduction
suppression
could
be
exerted
locally
and
was
mediated
by
preventing
the
formation
of
protein
tethers
necessary
for
current
activation
.
We
also
found
that
laminin-
332
could
locally
control
sensory
axon
branching
behavior
.
Loss
of
laminin-
332
in
humans
led
to
increased
sensory
terminal
branching
and
may
lead
to
a
de
-repression
of
mechanosensitive
currents
.
These
previously
unknown
functions
for
this
matrix
molecule
may
explain
some
of
the
extreme
pain
experienced
by
individuals
with
epidermolysis
bullosa
who
are
deficient
in
laminin-
332
.
Diseases
Validation
Diseases presenting
"a matrix that is inhibitory for sensory mechanotransduction"
symptom
junctional epidermolysis bullosa
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