The Journal of Neuroscience, April 11, 2007, ():
Anti-GM1 Antibodies Cause Complement-Mediated Disruption of Sodium Channel Clusters in Peripheral Motor Nerve Fibers
J. Neurosci. Susuki et al.
27: 3956
Supplemental Data
Files in this Data Supplement:
- supplemental material
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Supplemental figure 1.
Autoimmune attack caused by anti-GM1 IgG at internodal axons in ventral roots at the acute progressive phase.
A, GM1 and deposition of IgG. Both anti-GM1 mAb (GB2) (green, left column, cross section) and CT-B (green, right column, longitudinal section) bind to the affected axons with IgG deposition (red) (arrow). Asterisks indicate the axons with neither IgG nor GM1 staining.
B, IgG and complement deposition. Cross (left column) and longitudinal (right column) sections. Both IgG (red) and C3 (green) deposition are present on the axons. In some fibers, internodal axolemma is strongly stained (left column).
Scale bars: A, B, 10 μm.
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Supplemental figure 2.
Immunoreactivity of anti-GM1 IgG monoclonal antibody in control ventral root.
The axons run horizontally in all panels. The longitudinal sections of ventral root from control rabbit are immunostained with anti-GM1 mAb in green, and with Nav channels (A) or Kv channels (B) in red. GM1 staining is observed mainly at paranodal regions.
Scale bars: A, B, 10 μm.
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Supplemental figure 3.
Schematic presentation of the nodal disruption in peripheral motor nerve fibers in AMAN model.
A, Structures and molecular organizations at and near normal nodes of Ranvier. Nav channels are located at nodes and make multiprotein complexes including cytoskeletal proteins such as βIV spectrin. Nodal Nav channel clusters are further stabilized by interaction between the complexes and Schwann cell microvilli. Caspr forms axo-glial junction at paranodes, which act as diffusion barrier to restrict lateral mobility of nodal Nav channels. Kv channels are localized to juxtaparanodes.
B, Autoimmune-mediated disruption of Nav channel clusters and nodes of Ranvier. Anti-GM1 IgG antibodies cause complement-mediated attack with MAC formation at the nodal and paranodal axolemma. Nav channel clusters are altered by destruction of structures mediating their stabilization including: axonal cytoskeleton at nodes, Schwann cell microvilli, and paranodal junctions. As the autoimmune-mediated destruction extends, Nav channels and other components at and near nodes disappear. Kv channel clusters are preserved unless the immune attack extends to juxtaparanodes.
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Supplemental Table
