Reduced Survival of Motor Neuron (SMN) Protein in Motor Neuronal Progenitors Functions Cell Autonomously to Cause Spinal Muscular Atrophy in Model Mice Expressing the Human Centromeric (SMN2) Gene

Spinal muscular atrophy (SMA) is a common (∼1:6400) autosomal recessive neuromuscular disorder caused by a paucity of the survival of motor neuron (SMN) protein. Although widely recognized to cause selective spinal motor neuron loss when deficient, the precise cellular site of action of the SMN prot...

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Main Authors: Park, Gyu-Hwan, Maeno-Hikichi, Yuka, Awano, Tomoyuki, Landmesser, Lynn T., Monani, Umrao R.
Formato: Artigo
Idioma:Inglês
Publicado em: Society for Neuroscience 2010
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Articles
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2944776/
https://ncbi.nlm.nih.gov/pubmed/20826664
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1523/JNEUROSCI.2208-10.2010
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