Immunolocalisation of GQ1b and related gangliosides in human extraocular neuromuscular junctions and muscle spindles
2009 (English)In: Investigative Ophthalmology and Visual Science, ISSN 0146-0404, Vol. 50, no 7, 3226-3232 p.Article in journal (Refereed) Published
Purpose: To examine the distribution of anti-GQ1b, -GT1a and -GD1b antibody binding in human extraocular muscles (EOMs), axial and limb muscles and muscle spindles and thereby test the hypothesis that their distinctive ganglioside composition provides the molecular basis for selective involvement of EOMs and muscle spindles in Miller Fisher syndrome.
Methods: Muscle samples from adult human EOMs, vastus lateralis, biceps brachii, lumbrical, psoas and deep muscles of the neck were processed for immunohistochemistry, with monoclonal antibodies against ganglioside GQ1b, GT1a and GD1b. Neuromuscular junctions (NMJs) were detected by a-bungarotoxin binding and by acetycholinesterase reaction.
Results: The vast majority of motor endplates of human EOMs richly bound anti-GQ1b, -GT1a, and -GD1b ganglioside antibodies. Anti-GQ1b, -GT1a, and -GD1b ganglioside antibody bindings to NMJs in human limb and axial muscle were very scarce but the nerve terminals inside muscle spindles and in direct contact with intrafusal fibers were labeled with anti- GQ1b, -GT1a and -GD1b ganglioside antibodies.
Conclusions: The abundant and synaptic-specific binding of anti-GQ1b, -GT1a, and -GD1b ganglioside antibodies and the rich capillary supply in the human EOMs may partly explain the selective paralysis of these muscles in Miller Fisher syndrome.
Place, publisher, year, edition, pages
2009. Vol. 50, no 7, 3226-3232 p.
miller-fisher-syndrome; guillain-barre-syndrome; monoclonal-antibodies; igg antibody; transmission defect; anti-gq1b antibody; nervous-system; end-plates; ophthalmoplegia; expression
IdentifiersURN: urn:nbn:se:umu:diva-19309DOI: 10.1167/iovs.08-3333PubMedID: 19255160OAI: oai:DiVA.org:umu-19309DiVA: diva2:201699