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
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...
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
Download from
Any information missing or wrong?Report an Issue