Table 2.
ECM-related Schwann cell Phenotypes
| Mouse | Basal Lamina Appearance |
Radial Sorting Defect |
Myelin Phenotype | Remyelination | SC development and appearance |
Nodal Architecture |
References | |||
|---|---|---|---|---|---|---|---|---|---|---|
| Thinner | Thicker | Outfoldings | Stability | |||||||
| SC-specific β1 integrin knockout (P0-cre) | Disrupted; not tethered to SC cell surface | Yes; polyaxonal myelination | No | No | No | Normal | N.D. | Impaired differentiation; hypertrophic SC processes; normal proliferation and survival | N.D. | Feltri 2002 |
| Integrin α7 knockout | Normal | No | No | No | No | normal | N.D. | normal | N.D. | Previtalli 2003 |
| Integrin β4 knockout | N.D. | No | N.D. | N.D. | N.D. | N.D. | N.D. | N.D. | N.D. | Frei 1999 |
| SC-specific integrin β4 knockout | Normal | No | No | No | Yes in aged mice | Increased infoldings in aged mice | N.D. | normal | Normal; abnormal localiization of K channels | Nodari 2008 |
| SC-specific DG knockout (P0-cre) | Normal | Yes, but very mild; polyaxonal myelination | No | Yes | Yes | Spontaneous demyelination | N.D. | normal | Wider nodes; abnormal Nav clustering; disorganized SC microvilli (more severe than dy2J) | Saito 2003; Occhi 2005 |
| SC-specific integrin β4/DG double knockout (P0-cre) | Normal | No | Yes | No | Yes (more severe than DG KO) | Spontaneous demyelination (more severe than DG KO) | N.D. | Normal | Normal; abnormal localiization of K+ channels | Nodari 2008 |
| SC-specific FAK knockout (Cnp-cre) | Normal | Yes, severe; some sorting of large caliber axons | No | No | No | normal | N.D. | Reduced SC numbers; decreased SC proliferation; SC process outgrowth may not be impaired | N.D. | Grove 2007 |
| SC-specific Cdc42 KO (Dhh-cre) | Normal | Yes | No | No | No | Normal | N.D. | Decreased SC numbers; decreased SC proliferation; Impaired differentiation | N.D. | Benninger 2007 |
| SC-specific Rac1 KO (Dhh-cre) | Abnormal protrusions with empty loops | Yes; delayed | No | No | No | Normal | N.D. | Impaired SC process outgrowth and stability; Delayed differentiation; Normal SC numbers | N.D. | Benninger 2007 |
| SC-specific Rac1 KO (P0-Cre) | N.D. | Yes; delayed | Yes, partial | No | No | Normal | N.D. | Impaired ability to form SC lamellopodia; Delayed differentiation | N.D. | Nodari 2007 |
| SC-specific ILK KO (Dhh-cre) | Abnormal protrusions with empty loops | Yes; rescued by ROCK inhibitor | No | No | No | Normal | Impaired after nerve crush (independent of developmental defect) | Impaired SC process outgrowth and stability; Impaired SC differentiation; Normal SC numbers | N.D. | Pereira 2009 |
| Periaxin KO | Normal | No | No | Yes | Yes | Spontaneous demyelination | Impaired after nerve crush | N.D. (shorter internodes) | N.D. | Gillespie 2000 |