Abstract：
:Spinal muscular atrophy (SMA) is a lethal human disease characterized by motor neuron dysfunction and muscle deterioration due to depletion of the ubiquitous survival motor neuron (SMN) protein. Drosophila SMN mutants have reduced muscle size and defective locomotion, motor rhythm, and motor neuron neurotransmission. Unexpectedly, restoration of SMN in either muscles or motor neurons did not alter these phenotypes. Instead, SMN must be expressed in proprioceptive neurons and interneurons in the motor circuit to nonautonomously correct defects in motor neurons and muscles. SMN depletion disrupts the motor system subsequent to circuit development and can be mimicked by the inhibition of motor network function. Furthermore, increasing motor circuit excitability by genetic or pharmacological inhibition of K(+) channels can correct SMN-dependent phenotypes. These results establish sensory-motor circuit dysfunction as the origin of motor system deficits in this SMA model and suggest that enhancement of motor neural network activity could ameliorate the disease.
journal_name
Celljournal_title
Cellauthors
Imlach WL,Beck ES,Choi BJ,Lotti F,Pellizzoni L,McCabe BDdoi
10.1016/j.cell.2012.09.011subject
Has Abstractpub_date
2012-10-12 00:00:00pages
427-39issue
2eissn
0092-8674issn
1097-4172pii
S0092-8674(12)01114-2journal_volume
151pub_type
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