Rare Diseases Symptoms Automatic Extraction
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The RNA-binding protein FUS/TLS is a common aggregate-interacting protein in polyglutamine diseases.
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Neuronal
intranuclear
inclusions
(
NIIs
)
are
the
pathological
hallmark
of
polyglutamine
(
polyQ
)
diseases
.
We
previously
found
that
the
RNA-binding
protein
FUS
/
TLS
is
the
major
component
of
nuclear
polyQ
aggregates
of
a
cellular
model
of
Huntington
disease
.
In
this
study
,
we
revealed
that
FUS
/
TLS
binds
to
NIIs
in
the
human
brains
from
patients
with
spinocerebellar
ataxia
type
1
,
2
,
3
,
and
dentatorubral-pallidoluysian
atrophy
.
Recent
reports
have
revealed
that
mutations
in
FUS
/
TLS
gene
are
responsible
for
familial
amyotrophic
lateral
sclerosis
6
(
ALS
6
)
.
Our
results
indicated
that
changing
FUS
/
TLS
to
an
insoluble
form
may
be
a
common
process
in
polyQ
diseases
and
ALS
6
.
