Dysregulation of innate immune signaling in animal models of Spinal Muscular Atrophy
Eric L. Garcia,
Rebecca E. Steiner,
Amanda C. Raimer
et al.
Abstract:BackgroundSpinal Muscular Atrophy (SMA) is a devastating neuromuscular disease caused by hypomorphic loss of function in the Survival Motor Neuron (SMN) protein. SMA presents across broad spectrum of disease severity. Unfortunately, vertebrate models of intermediate SMA have been difficult to generate and are thus unable to address key aspects of disease etiology. To address these issues, we developed aDrosophilamodel system that recapitulates the full range of SMA severity, allowing studies of pre-onset biolo… Show more
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