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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
"sensory axonal neuropathy"
symptom
gm1 gangliosidosis
triple a syndrome
This symptom has already been validated