| First Author | Ruiz M | Year | 2014 |
| Journal | Neuropharmacology | Volume | 83 |
| Pages | 137-53 | PubMed ID | 24769002 |
| Mgi Jnum | J:215345 | Mgi Id | MGI:5605139 |
| Doi | 10.1016/j.neuropharm.2014.04.009 | Citation | Ruiz M, et al. (2014) Neurodegenerative changes are prevented by Erythropoietin in the pmn model of motoneuron degeneration. Neuropharmacology 83:137-53 |
| abstractText | Motoneuron diseases are fatal neurodegenerative disorders characterized by a progressive loss of motoneurons, muscle weakness and premature death. The progressive motor neuronopathy (pmn) mutant mouse has been considered a good model for the autosomal recessive childhood form of spinal muscular atrophy (SMA). Here, we investigated the therapeutic potential of Erythropoietin (Epo) on this mutant mouse. Symptomatic or pre-symptomatic treatment with Epo significantly prolongs lifespan by 84.6% or 87.2% respectively. Epo preserves muscle strength and significantly attenuates behavioural motor deficits of mutant pmn mice. Histological and metabolic changes in the spinal cord evaluated by immunohistochemistry, western blot, and high-resolution (1)H-NMR spectroscopy were also greatly prevented by Epo-treatment. Our results illustrate the efficacy of Epo in improving quality of life of mutant pmn mice and open novel therapeutic pathways for motoneuron diseases. |
