Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Dysfunction of nodes of Ranvier: a mechanism for anti-ganglioside antibody-mediated neuropathies.
[gm1 gangliosidosis]
Autoantibodies
against
gangliosides
GM
1
or
GD
1
a
are
associated
with
acute
motor
axonal
neuropathy
(
AMAN
)
and
acute
motor
-
sensory
axonal
neuropathy
(
AMSAN
)
,
whereas
antibodies
to
GD
1
b
ganglioside
are
detected
in
acute
sensory
ataxic
neuropathy
(
ASAN
)
.
These
neuropathies
have
been
proposed
to
be
closely
related
and
comprise
a
continuous
spectrum
,
although
the
underlying
mechanisms
,
especially
for
sensory
nerve
involvement
,
are
still
unclear
.
Antibodies
to
GM
1
and
GD
1
a
have
been
proposed
to
disrupt
the
nodes
of
Ranvier
in
motor
nerves
via
complement
pathway
.
We
hypothesized
that
the
disruption
of
nodes
of
Ranvier
is
a
common
mechanism
whereby
various
anti-ganglioside
antibodies
found
in
these
neuropathies
lead
to
nervous
system
dysfunction
.
Here
,
we
show
that
the
IgG
monoclonal
anti-
GD
1
a
/
GT
1
b
antibody
injected
into
rat
sciatic
nerves
caused
deposition
of
IgG
and
complement
products
on
the
nodal
axolemma
and
disrupted
clusters
of
nodal
and
paranodal
molecules
predominantly
in
motor
nerves
,
and
induced
early
reversible
motor
nerve
conduction
block
.
Injection
of
IgG
monoclonal
anti-
GD
1
b
antibody
induced
nodal
disruption
predominantly
in
sensory
nerves
.
In
an
ASAN
rabbit
model
associated
with
IgG
anti-
GD
1
b
antibodies
,
complement-mediated
nodal
disruption
was
observed
predominantly
in
sensory
nerves
.
In
an
AMAN
rabbit
model
associated
with
IgG
anti-
GM
1
antibodies
,
complement
attack
of
nodes
was
found
primarily
in
motor
nerves
,
but
occasionally
in
sensory
nerves
as
well
.
Periaxonal
macrophages
and
axonal
degeneration
were
observed
in
dorsal
roots
from
ASAN
rabbits
and
AMAN
rabbits
.
Thus
,
nodal
disruption
may
be
a
common
mechanism
in
immune-mediated
neuropathies
associated
with
autoantibodies
to
gangliosides
GM
1
,
GD
1
a
,
or
GD
1
b
,
providing
an
explanation
for
the
continuous
spectrum
of
AMAN
,
AMSAN
,
and
ASAN
.
Diseases
Validation
Diseases presenting
"common mechanism"
symptom
canavan disease
familial hypocalciuric hypercalcemia
focal myositis
gm1 gangliosidosis
waldenström macroglobulinemia
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom