Table 1.
Comparison between early and late pathological stages in the lower motor system of TWI mice compared with WT littermates (−absence of signs; +mild differences; ++medium differences; +++severe differences).
| P15 | P30 | |
|---|---|---|
| SPINAL CORD | ||
| MN bodies | ||
| number | − | − |
| size | − | − |
| shape | − | − |
| abnormal distribution of cytoskeleton | − | + |
| lytic organelles | − | + |
| subcellular compartments | − | − |
| Ependyma channel | ||
| cell-to-cell junctions | − | +++ |
| Microvilli | − | +++ |
| Cilia | − | − |
| nuclear envelope | − | +++ |
| Blood vessel | ||
| thickness’ discontinouty | − | ++ |
| intraluminal protrusion | − | + |
| SCIATIC NERVE | ||
| Nerve morphology | ||
| swelling of nerve diameter | ++ | +++ |
| infiltration of globoid cells | ++ | +++ |
| scar tissue | ++ | ++ |
| enlarged cytosol | + | +++ |
| empty spaces | + | +++ |
| Remak bundles | +++ | +++ |
| Myelinated axons morphometry | ||
| diameter | + | +++ |
| area | ++ | +++ |
| increased cytoskeletal density | + | +++ |
| myelin sheaths thickness | + | +++ |
| Multiple myelination process | ||
| type 1 | + | +++ |
| type 2 | − | ++ |
| type 3 | − | + |
| GASTROCNEMIUS | ||
| Fibers | ||
| fiber diameter | +++ | +++ |
| alterated mitochondria | + | +++ |
| alterated sarcoplasmic reticulum | − | +++ |
| NMJ | ||
| type 1 | 50% | 27% |
| type 2 | 40% | 33% |
| type 3 | 10% | 40% |