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Monospecific high-affinity and complement activating anti-GM1 antibodies are determinants in experimental axonal neuropathy.
[gm1 gangliosidosis]
It
has
been
difficult
to
replicate
consistently
the
experimental
model
of
axonal
Guillain-
Barré
syndrome
(
GBS
)
.
We
immunized
rabbits
with
two
lipo-oligosaccharides
(
LOS
1
and
LOS
2
)
derived
from
the
same
C
.
jejuni
strain
and
purified
in
a
slightly
different
way
.
LOS
1
did
not
contain
proteins
whereas
several
proteins
were
present
in
LOS
2
.
In
spite
of
a
robust
anti-
GM
1
antibody
response
in
all
animals
the
neuropathy
developed
only
in
rabbits
immunized
with
LOS
1
.
To
explain
this
discrepancy
we
investigated
fine
specificity
,
affinity
and
ability
to
activate
the
complement
of
anti-
GM
1
antibodies
.
Only
rabbits
immunized
with
LOS
1
showed
monospecific
high
-affinity
antibodies
which
activated
more
effectively
the
complement
.
Although
it
is
not
well
understood
how
monospecific
high
-affinity
antibodies
are
induced
these
are
crucial
for
the
induction
of
experimental
axonal
neuropathy
.
Only
a
strict
adherence
to
the
protocols
demonstrated
to
be
successful
may
guarantee
the
reproducibility
and
increase
the
confidence
in
the
animal
model
as
a
reliable
tool
for
the
study
of
the
human
axonal
GBS
.
Diseases
Validation
Diseases presenting
"neuropathy"
symptom
adrenomyeloneuropathy
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
fabry disease
focal myositis
gm1 gangliosidosis
hirschsprung disease
homocystinuria without methylmalonic aciduria
inclusion body myositis
krabbe disease
malignant atrophic papulosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
neuralgic amyotrophy
oculocutaneous albinism
pendred syndrome
phenylketonuria
pyomyositis
pyruvate dehydrogenase deficiency
sneddon syndrome
thoracic outlet syndrome
triple a syndrome
von hippel-lindau disease
waldenström macroglobulinemia
wolf-hirschhorn syndrome
This symptom has already been validated