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David Borchelt & Jacob Ayers

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David Borchelt, PhD & Jacob Ayers, PhD

University of Florida & 

Role of misfoldedwild-type SOD1 in cases of sporadic ALS

Mutations in SOD1 have been linked to familial ALS, and we intend to investigate the potential that some cases of sporadic ALS may be caused by the accumulation of misfolded SOD1. In prior studies funded by Packard, we have created a bioassay for the presence of misfolded SOD1 that injects spinal tissue preparations into the spinal column of mice that express a reporter protein, which is a version of SOD1 fused to yellow fluorescent protein (YFP). Some types of misfolded SOD1 induce misfolding in otherwise normal SOD1 proteins. If this occurs in recipient mice, we will know the injected samples contain misfolded SOD1. We intend to prepare spinal tissue extracts from autopsied sporadic ALS patients to determine whether these tissues may contain misfolded SOD1.

 

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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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