Rare Diseases Symptoms Automatic Extraction
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A random Abstract
Our Project
Our Team
WIP modulates dendritic spine actin cytoskeleton by transcriptional control of lipid metabolic enzymes.
[wiskott-aldrich syndrome]
We
identify
Wiskott-
Aldrich
syndrome
protein
(
WASP
)
-
interacting
protein
(
WIP
)
as
a
novel
component
of
neuronal
synapses
whose
absence
increases
dendritic
spine
size
and
filamentous
actin
levels
in
an
N-WASP
/
Arp
2
/
3
-
independent
,
RhoA
/
ROCK
/
profilinIIa-dependent
manner
.
These
effects
depend
on
the
reduction
of
membrane
sphingomyelin
(
SM
)
due
to
transcriptional
upregulation
of
neutral
sphingomyelinase
(
NSM
)
through
active
RhoA
;
this
enhances
RhoA
binding
to
the
membrane
,
raft
partitioning
and
activation
in
steady
state
but
prevents
RhoA
changes
in
response
to
stimulus
.
Inhibition
of
NSM
or
SM
addition
reverses
RhoA
,
filamentous
actin
and
functional
anomalies
in
synapses
lacking
WIP
.
Our
findings
characterize
WIP
as
a
link
between
membrane
lipid
composition
and
actin
cytoskeleton
at
dendritic
spines
.
They
also
contribute
to
explain
cognitive
deficits
shared
by
individuals
bearing
mutations
in
the
region
assigned
to
the
gene
encoding
for
WIP
.