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Zuoshang Xu, PhD

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Zuoshang Xu, PhD

University of Massachusetts

How interneuron expression of mutant PFN1 drives motor neuron degeneration and clinical progression of ALS

Spinal cord interneurons connect with motor neurons and they modulate and regulate motor neuron activities. Previous studies in animal models and in humans suggest that interneurons degenerate along with motor neurons in ALS, but how interneuron degeneration affect motor neuron degeneration and clinical disease progression has not been defined. We have generated transgenic mouse models for ALS where the role of interneurons in ALS can be analyzed. We propose to take advantage of this model and define the role of interneurons in ALS.

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Steven Finkbeiner

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Gladstone Institutes, UCSF
Two recently discovered genes that have been associated with both familial and sporadic forms of ALS encode the related proteins TDP43 and FUS cause neuron death in ALS.
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