Article
Inhibition of autophagy delays motoneuron degeneration and extends lifespan in a mouse model of spinal muscular atrophy.
Cell death & disease - 20 Dec 2017
Piras Antonio, Schiaffino Lorenzo, Boido Marina, Valsecchi Valeria, Guglielmotto Michela, De Amicis Elena, Puyal Julien, Garcera Ana, Tamagno Elena, Soler Rosa M, Vercelli Alessandro
Abstract excerpt
Spinal muscular atrophy (SMA) is a recessive autosomal neuromuscular disease, due to homozygous mutations or deletions in the telomeric survival motoneuron gene 1 (SMN1). SMA is characterized by motor impairment, muscle atrophy, and premature death following motor neuron (MN) degeneration. Emerging evidence suggests that dysregulation of autophagy contributes to MN degeneration. We here investigated the role of...
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