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Neuropharmacology ; 83: 137-53, 2014 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-24769002

RESUMO

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.


Assuntos
Eritropoetina/uso terapêutico , Neurônios Motores/efeitos dos fármacos , Medula Espinal/efeitos dos fármacos , Atrofias Musculares Espinais da Infância/tratamento farmacológico , Animais , Modelos Animais de Doenças , Camundongos , Camundongos Mutantes , Atividade Motora/efeitos dos fármacos , Receptores da Eritropoetina/metabolismo , Medula Espinal/metabolismo
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