Article
A Drosophila melanogaster model of spinal muscular atrophy reveals a function for SMN in striated muscle.
The Journal of cell biology - 12 Mar 2007
Rajendra T K, Gonsalvez Graydon B, Walker Michael P, Shpargel Karl B, Salz Helen K, Matera A Gregory
Abstract excerpt
Mutations in human survival motor neurons 1 (SMN1) cause spinal muscular atrophy (SMA) and are associated with defects in assembly of small nuclear ribonucleoproteins (snRNPs) in vitro. However, the etiological link between snRNPs and SMA is unclear. We have developed a Drosophila melanogaster system to model SMA in vivo. Larval-lethal Smn-null mutations show no detectable snRNP reduction, making it unlikely that...
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