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Cellular and ultra structural evidence for cytoskeletal localization of prolyl endopeptidase-like protein in neurons.
[cystinuria]
The
biochemical
properties
and
subcellular
localization
of
prolyl
endopeptidase
(
PREP
)
in
brain
are
well
characterized
and
its
implications
in
the
realization
of
cognitive
processes
and
in
the
pathogenesis
of
neurodegenerative
disorders
are
a
matter
of
intensive
investigation
.
In
contrast
,
very
little
is
known
about
its
homolog
,
the
PREP
-like
protein
(
PREPL
)
.
In
order
to
obtain
initial
hints
about
the
involvement
of
PREPL
in
physiological
processes
,
a
differential
proteomic
screen
was
performed
with
human
skin
fibroblasts
from
controls
and
patients
with
PREPL
deficiency
(
hypotonia
-
cystinuria
syndrome
)
.
The
majority
of
affected
proteins
represented
cytoskeletal
proteins
,
including
caldesmon
,
tropomyosin
α
3
chain
,
lamin
A
,
β-actin
,
γ-actin
,
vimentin
and
zyxin
.
Therefore
,
the
analysis
of
PREPL
subcellular
localization
by
confocal
laser
scanning
and
electron
microscopy
in
mouse
neurons
was
focused
on
the
cytoskeleton
.
The
co
-localization
of
PREPL
with
cytoskeletal
marker
proteins
such
as
β-actin
and
microtubulin-associated
protein-
2
was
observed
,
in
addition
to
the
presence
of
PREPL
within
Golgi
apparatus
and
growth
cones
.
In
the
mouse
brain
,
PREPL
is
neuronally
expressed
and
highly
abundant
in
neocortex
,
substantia
nigra
and
locus
coeruleus
.
This
mirrors
to
some
extent
the
distribution
pattern
of
PREP
and
points
toward
redundant
functions
of
both
proteins
.
In
the
human
neocortex
,
PREPL
immunostaining
was
found
in
the
cytoplasm
and
in
neuropil
,
in
particular
of
layer
V
pyramidal
neurons
.
This
staining
was
reduced
in
the
neocortex
of
Alzheimer
's
disease
(
AD
)
patients
.
Moreover
,
in
AD
brains
,
PREPL
immunoreactivity
was
observed
in
the
nucleus
and
in
varicose
neuritic
processes
.
Our
data
indicate
physiological
functions
of
PREPL
associated
with
the
cytoskeleton
,
which
may
be
affected
under
conditions
of
cytoskeletal
degeneration
.
Diseases
Validation
Diseases presenting
"pyramidal neurons"
symptom
cystinuria
hereditary cerebral hemorrhage with amyloidosis
hirschsprung disease
inclusion body myositis
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