Article
Axon outgrowth and neuronal differentiation defects after a-SMN and FL-SMN silencing in primary hippocampal cultures.
PloS one - 1 Jan 2018
Pletto Daniela, Capra Silvia, Finardi Adele, Colciaghi Francesca, Nobili Paola, Battaglia Giorgio Stefano, Locatelli Denise, Cagnoli Cinzia
Abstract excerpt
Spinal Muscular Atrophy (SMA) is a severe autosomal recessive disease characterized by selective motor neuron degeneration, caused by disruptions of the Survival of Motor Neuron 1 (Smn1) gene. The main product of SMN1 is the full-length SMN protein (FL-SMN), that plays an established role in mRNA splicing. FL-SMN is also involved in neurite outgrowth and axonal transport. A shorter SMN isoform, axonal-SMN or...
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