The Journal of Neuroscience, February 9, 2005, ():
Altered Ion Channels in an Animal Model of Charcot-Marie-Tooth Disease Type IA
J. Neurosci. Devaux and Scherer
25: 1470
Supplemental data
Files in this Data Supplement:
- supplemental material
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Supplemental Figure 1: EBP-50 in |*bital*|Trembler-J|*eital*| and WT sciatic nerve.
These are fixed teased sciatic nerves fibers from P3, P10 and 3 months old WT mice, and 3 months old |*bital*|Trembler-J|*eital*| mice immunostained as indicated.
EBP-50 clusters at node vicinity as early as P3 and P10, almost every node or hemi-node is EBP-50-positive. By contrast in |*bital*|Trembler-J|*eital*| mice, many (about 40%) of nodes are EBP-50-positive. Scale bars: 10 |*mu*|m.
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Supplemental Figure 2: Syndecan-3 in |*bital*|Trembler-J|*eital*| and WT sciatic nerve.
These are fixed teased sciatic nerves fibers from 3 months old WT mice, and |*bital*|Trembler-J|*eital*| mice immunostained as indicated. Whereas syndecan-3 is present at all nodes in WT nerves, it is reduced at |*bital*|Trembler-J|*eital*| nodes. Scale bars: 10 |*mu*|m.
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Supplemental Figure 3: Western blot of Kv3.1b, Kv1.1, and Nav1.8.
These are western blot of spinal cord (Spcd; 25 |*mu*|g in A-B; 50 |*mu*|g in C) and sciatic nerve (100 |*mu*|g) membrane from WT and |*bital*|Trembler-J|*eital*| mice probed for Kv3.1b (A), Kv1.1 (B) and Nav1.8 (C). No bands corresponding to Kv3.1b, Kv1.1, or Nav1.8 are detected in the sciatic nerve extract of WT or |*bital*|Trembler-J|*eital*| mice. However, bands were detected at the correct molecular weight (~97 kDa for Kv3.1b; ~80 kDa for Kv1.1; ~245 kD for Nav1.8) in the spinal cord extract from both WT and |*bital*|Trembler-J|*eital*| mice.
