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Molecular markers for granulovacuolar degeneration are present in rimmed vacuoles.
[inclusion body myositis]
Rimmed
vacuoles
(
RVs
)
are
round
-oval
cytoplasmic
inclusions
,
detected
in
muscle
cells
of
patients
with
myopathies
,
such
as
inclusion
body
myositis
(
IBM
)
and
distal
myopathy
with
RVs
(
DMRV
)
.
Granulovacuolar
degeneration
(
GVD
)
bodies
are
spherical
vacuoles
containing
argentophilic
and
hematoxyphilic
granules
,
and
are
one
of
the
pathological
hallmarks
commonly
found
in
hippocampal
pyramidal
neurons
of
patients
with
aging-related
neurodegenerative
diseases
,
such
as
Alzheimer
's
disease
and
Parkinson
's
disease
.
These
diseases
are
common
in
the
elderly
and
share
some
pathological
features
.
Therefore
,
we
hypothesized
that
mechanisms
of
vacuolar
formation
in
RVs
and
GVD
bodies
are
common
despite
their
role
in
two
differing
pathologies
.
We
explored
the
components
of
RVs
by
immunohistochemistry
,
using
antibodies
for
GVD
markers
.
S
ubjects
included
one
AD
case
,
eight
cases
of
sporadic
IBM
,
and
three
cases
of
DMRV
.
We
compared
immunoreactivity
and
staining
patterns
for
GVD
markers
.
These
markers
included
:
(
1
)
tau-modifying
proteins
(
caspase
3
,
cyclin-dependent
kinase
5
[
CDK
5
]
,
casein
kinase
1
δ
[
CK
1
δ
]
,
and
c-jun
N-
terminal
kinase
[
JNK
]
)
,
(
2
)
lipid
raft-associated
materials
(
annexin
2
,
leucine-rich
repeat
kinase
2
[
LRRK
2
]
,
and
flotillin-
1
)
,
and
(
3
)
other
markers
(
charged
multi-vesicular
body
protein
2
B
[
CHMP
2
B
]
and
phosphorylated
transactive
response
DNA
binding
protein-
43
[
pTDP
43
]
)
in
both
GVD
bodies
and
RVs
.
Furthermore
,
we
performed
double
staining
of
each
GVD
marker
with
pTDP
43
to
verify
the
co
-localization
.
GVD
markers
,
including
lipid
raft-associated
proteins
and
tau
kinases
,
were
detected
in
RVs
.
CHMP
2
B
,
pTDP
43
,
caspase
3
,
LRRK
2
,
annexin
2
and
flotillin-
1
were
detected
on
the
rim
and
were
diffusely
distributed
in
the
cytoplasm
of
RV-
positive
fibers
.
CDK
5
,
CK
1
δ
and
JNK
were
detected
only
on
the
rim
.
In
double
staining
experiments
,
all
GVD
markers
colocalized
with
pTDP
43
in
RVs
.
These
results
suggest
that
RVs
of
muscle
cells
and
GVD
bodies
of
neurons
share
a
number
of
molecules
,
such
as
raft-related
proteins
and
tau-modifying
proteins
.
Diseases
Validation
Diseases presenting
"granulovacuolar degeneration"
symptom
inclusion body myositis
This symptom has already been validated