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Article

Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophy

20 May 2008

Abstract excerpt

Spinal muscular atrophy (SMA) is a common pediatric neuromuscular disorder caused by insufficient levels of the survival of motor neuron (SMN) protein. Studies involving SMA patients and animal models expressing the human SMN2 gene have yielded relatively little information about the earliest cellular consequences of reduced SMN protein. In this study, we have used severe- and mild-SMN2 expressing mouse models of...

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PMID: 18492800
DOI: 10.1093/hmg/ddn156

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