Article
The spinal muscular atrophy mouse model, SMAΔ7, displays altered axonal transport without global neurofilament alterations.
Acta neuropathologica - 1 Sept 2011
Dale Jeffrey M, Shen Hailian, Barry Devin M, Garcia Virginia B, Rose Ferrill F, Lorson Christian L, Garcia Michael L
Abstract excerpt
Spinal muscular atrophy (SMA) is a neurodegenerative disease resulting from decreased levels of survival motor neuron 1 (SMN1) protein. Reduced SMN1 levels are linked to pathology at neuromuscular junctions (NMJs), which includes decreased vesicle density and organization, decreased quantal release, increased endplate potential duration, and neurofilament (NF) accumulations. This work presents a first study...
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