Table 4.
List of therapeutic approaches tested on wobbler mice
| Target | Compound(s) | Effect on WR MND | Reference |
|---|---|---|---|
| Trophic factors neuroprotection | BDNF (brain-derived neurotrophic factor) | Beneficial, delayed motor impairment, inhibition of NOS activity | (Mitsumoto et al. 1994; Tsuzaka et al. 2001) |
| CTNF (ciliary neurotrophic factor) | Beneficial, delayed motor impairment | (Mitsumoto et al. 1994) | |
| BDNF + CTNF | Beneficial, delayed motor impairment | (Mitsumoto et al. 1994) | |
| Human recombinant IGF (insulin-like growth factor) | Beneficial, delayed motor impairment, increase of body weight | (Ikeda et al. 1995a) | |
| IGF + GAG (glycosaminoglycans) | Beneficial, delayed motor impairment, increase of body weight | (Gorio et al. 1998; Vergani et al. 1999) | |
| CT-1 (cardiotrophin-1) | Beneficial, delayed motor impairment | (Mitsumoto et al. 2001) | |
| Antiinflammatory agents | LIF (leukaemia inhibitory factor) | Beneficial, increased muscle strength | (Ikeda et al. 1995a) |
| IL-6 (interleukin-6)+ soluble IL6 receptor | Beneficial, delayed motor impairment | (Ikeda et al. 1996) | |
| Human tumor necrosis factor (TNF) binding protein 1 | Beneficial, delayed motor impairment | (Bigini et al. 2008) | |
| VB3323, a TLR4 (Toll-like receptor 4) antagonist | Beneficial, delayed motor impairment, decreased microglia activation | (Fumagalli et al. 2006; De Paola et al. 2012) | |
| EPO (erythropoietin) | Beneficial, improved motor performance, decreased astro- and microgliosis | (Mennini et al. 2006) | |
| Antiglutamatergic agents | Riluzole | Beneficial, delayed motor impairment | (Ishiyama et al. 2004) |
| MK801, a NMDA receptor antagonist | No beneficial effects | (Krieger et al. 1992) | |
| RPR119990, an AMPA receptor antagonist | No beneficial effects | (Fumagalli et al. 2006) | |
| Mitochondrial support and antioxidant agents | Creatine monohydrate | Beneficial, improved motor performance | (Ikeda et al. 2000a, b) |
| HBOT (hyperbaric oxygen therapy) | Beneficial, improved motor performance | (Dave et al. 2003a, b) | |
| ALCAR (acetyl-l-carnitine | No beneficial effects | (Bigini et al. 2008; Beghi et al. 2011) | |
| OPC-14117 a free radical scavenger | Beneficial, improved motor performance, decreased formation of lipid peroxides | (Abe et al. 1997) | |
| Lecithinized superoxide dismutase | Beneficial, improved motor performance | (Ikeda et al. 1995b) | |
| l-NAME, a nonselective NOS inhibitor | Beneficial, improved motor performance | (Ikeda et al. 1998) | |
| Steroid hormones | Progesterone | Beneficial, improved motor performance | (Gonzalez Deniselle et al. 2002; Deniselle et al. 2012) |
| U-74389F, a 21-amino-steroid | Beneficial, improved motor performance and decreased gliosis | (Gonzalez Deniselle et al. 1996) | |
| Stem cell therapy | Human cord blood mononuclear cells (injected in the brain ventricle) | Beneficial, improved motor performance, but did not reach the spinal cord | (Bigini et al. 2011) |
| Human skeletal muscle-derived stem cells (injected in the brain ventricle) | Beneficial, improved motor performance, but did not reach the spinal cord | (Canzi et al. 2012) | |
| Human amniotic fluid cells (injected in the brain ventricle) | No or mild beneficial effect on motor performance no effect on survival | (Bigini et al. 2012) |