Grants

Alec Smith and NDRG team awarded NIH R21 grant

Alec Smith is the PI of a newly awarded NIH R21 grant to study muscle spindle dysfunction in Duchenne Muscular Dystrophy. David Mack, Mike Regnier, and Mark Bothwell are co-investigators.

Muscle spindles are sensory end organs in muscle that allow sensation of muscle position and movement. As muscle spindles are comprised of clusters of specialized types of muscle fibers, their proper function is likely dependent on dystrophin, just as the muscle proper is. Dystrophin mutations in Duchenne Muscular Dystrophy cause a loss of muscle cell dystrophin. Thus, it is plausible that DMD patients may experience muscle spindle dysfunction, which may contribute to the tendency of DMD patients to suffer frequent falls.

In order to develop an experimental system to study human muscle spindle function, Alec and team will produce muscle spindle cells in vitro, by differentiation of induced pluripotent stem cells bearing a dystrophin mutation, and from wild type control cells.

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Alec Smith awarded NIH R03 grant

Alec Smith has been awarded a two-year R03 grant from NIH entitled Using functional readouts from engineering models of innervated skeletal muscle to assess the efficacy of CRISPR-based c9orf72 ALS gene therapies.

This project is part of a larger effort to employ human induced pluripotent stem cell-derived motor neurons and skeletal muscle to produce in vitro models of the neuromuscular junction to model motor neuron diseases in order to study disease mechanisms and for drug screens.

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ISCRM Innovation Pilot Award to Alec Smith

Alec Smith has been awarded an Innovation Pilot Award by ISCRM. This Washington-State-funded one year grant will fund research into the effect of Duchenne muscular dystrophy on muscle spindles, the sensory receptor structures within skeletal muscle that allow us to sense the extent and speed of contraction of our muscles to permit coordinated movement.

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