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