Overexpression of survival motor neuron improves neuromuscular function and motor neuron survival in mutant SOD1 mice.

Journal article

Spinal muscular atrophy results from diminished levels of survival motor neuron (SMN) protein in spinal motor neurons. Low levels of SMN also occur in models of amyotrophic lateral sclerosis (ALS) caused by mutant superoxide dismutase 1 (SOD1) and genetic reduction of SMN levels exacerbates the phenotype of transgenic SOD1(G93A) mice. Here, we demonstrate that SMN protein is significantly reduced in the spinal cords of patients with sporadic ALS. To test the potential of SMN as a modifier of ...

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Authors: Turner, B; Alfazema, N; Sheean, R; Sleigh, J; Davies, K

• Publication status: Published
• Journal: Neurobiology of aging
• Volume: 35
• Issue: 4
• Pages: 906-915
• Publication date: 2014-04-01
• DOI: 10.1016/j.neurobiolaging.2013.09.030
• EISSN: 1558-1497
• ISSN: 0197-4580
• Language: English
• Keywords: Superoxide Dismutase; Adult; Muscular Atrophy, Spinal; Aged; Animals; Gene Expression; Female; Male; Mutation; Mice, Transgenic; Humans; Spinal Cord; Survival of Motor Neuron 1 Protein; Cell Survival; Motor Neurons; Up-Regulation; Aged, 80 and over; Middle Aged; Disease Models, Animal; Amyotrophic Lateral Sclerosis; Motor Activity