6533b85bfe1ef96bd12bbeb2

RESEARCH PRODUCT

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

Ana Fe Martínez-vidalDaniel MonleonMarta RuizJosé Manuel MoralesMinerva Giménez Y Ribotta

subject

medicine.medical_specialtyMutantMotor ActivitySpinal Muscular Atrophies of ChildhoodMiceCellular and Molecular NeuroscienceWestern blotInternal medicineReceptors ErythropoietinmedicineAnimalsErythropoietinMotor NeuronsPharmacologymedicine.diagnostic_testbusiness.industryMuscle weaknessSpinal muscular atrophymedicine.diseaseSpinal cordSMA*Mice Mutant StrainsDisease Models Animalmedicine.anatomical_structureEndocrinologySpinal CordErythropoietinImmunohistochemistrymedicine.symptombusinessNeurosciencemedicine.drug

description

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.

yearjournalcountryeditionlanguage
2014-08-01Neuropharmacology
https://doi.org/10.1016/j.neuropharm.2014.04.009