Neurodegenerative changes are prevented by Erythropoietin in the pmn model of motoneuron degeneration

Authors
M. Ruiz, A. F. Martínez-Vidala J. M. Morales, D. Monleón, M. Giménez et al.


Lab
Universidad Miguel Hernández (UMH), San Juan de Alicante, Spain

Journal
Neuropharmacology

Abstract
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 1H-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.

BIOSEB Instruments Used
Grip strength test (BIO-GS3)

Source :

http://www.sciencedirect.com/science/article/pii/S002839081400135X

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