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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
"pain"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
adrenomyeloneuropathy
aniridia
aromatase deficiency
carcinoma of the gallbladder
cholangiocarcinoma
coats disease
congenital diaphragmatic hernia
congenital toxoplasmosis
cushing syndrome
cutaneous mastocytosis
cystinuria
dedifferentiated liposarcoma
dentin dysplasia
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
focal myositis
hirschsprung disease
hodgkin lymphoma, classical
holt-oram syndrome
junctional epidermolysis bullosa
kabuki syndrome
kindler syndrome
lamellar ichthyosis
liposarcoma
locked-in syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
neuralgic amyotrophy
oligodontia
oral submucous fibrosis
papillon-lefèvre syndrome
phenylketonuria
pleomorphic liposarcoma
primary hyperoxaluria type 1
proteus syndrome
pyomyositis
scrub typhus
sneddon syndrome
systemic capillary leak syndrome
thoracic outlet syndrome
trochlear dysplasia
typhoid
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
wolf-hirschhorn syndrome
This symptom has already been validated