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Transcription profiling of mouse spinal chord from a mouse model of severe spinal muscular atrophy


ABSTRACT: Pre-symptomatic development of lower motor neuron connectivity in a mouse model of severe spinal muscular atrophy

ORGANISM(S): Mus musculus

DISEASE(S): spinal muscular atrophy

SUBMITTER: Sheena Lee 

PROVIDER: E-MEXP-2428 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Pre-symptomatic development of lower motor neuron connectivity in a mouse model of severe spinal muscular atrophy.

Murray Lyndsay M LM   Lee Sheena S   Bäumer Dirk D   Parson Simon H SH   Talbot Kevin K   Gillingwater Thomas H TH  

Human molecular genetics 20091102 3


The childhood motor neuron disease spinal muscular atrophy (SMA) results from reduced expression of the survival motor neuron (SMN) gene. Previous studies using in vitro model systems and lower organisms have suggested that low levels of Smn protein disrupt prenatal developmental processes in lower motor neurons, influencing neuronal outgrowth, axon branching and neuromuscular connectivity. The extent to which these developmental pathways contribute to selective vulnerability and pathology in th  ...[more]

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