publication . Doctoral thesis . 2016

Atròfia muscular espinal: mecanismes patogènics i estratègies terapèutiques en models murins de la malaltia

Cerveró Cebrià, Clàudia;
Open Access Catalan; Valencian
  • Published: 16 Sep 2016
  • Publisher: Universitat de Lleida
Abstract
The spinal muscular atrophy (SMA) is a genetic disease that affects spinal motor neurons causing its death and muscle atrophy. This study is divided in three parts. First. Characterization of the Smn2B/- mice, a mild SMA phenotype model. A multisistemic affectation was reported to accompany the better known neuromuscular alteration in these mice. Second. Study of C-type cholinergic synapses in the SMA and therapeutic trial with the sigma-1 receptor agonist PRE-084 (molecule present in C boutons) in the SMNΔ7 and Smn2B/- mice. Although PRE-084 did not confer neuroprotection, it prevented the SMA characteristic reactive gliosis. Third. Treatment with AICAR, an exe...
Subjects
free text keywords: Atròfia muscular espinal, Models murins, Sinapsis neuromuscular, Motoneurona, Múscul esquelètic, Agents terapèutics, Spinal muscular atrophy, Mouse models, Neuromuscular junction, Motoneuron, Skeletal muscle, Therapeutic agents, Medicina, 616.8
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