| Chapter title |
Intercellular Prion-Like Conversion and Transmission of Cu/Zn Superoxide Dismutase (SOD1) in Cell Culture
|
|---|---|
| Chapter number | 24 |
| Book title |
Prions
|
| Published in |
Methods in molecular biology, January 2017
|
| DOI | 10.1007/978-1-4939-7244-9_24 |
| Pubmed ID | |
| Book ISBNs |
978-1-4939-7242-5, 978-1-4939-7244-9
|
| Authors |
Leslie I. Grad, Edward Pokrishevsky, Neil R. Cashman, Grad, Leslie I., Pokrishevsky, Edward, Cashman, Neil R. |
| Abstract |
The prion hypothesis has extended to the fatal motor neuron disease, amyotrophic lateral sclerosis (ALS), as a means to explain the spatiotemporal spread of pathology from one or more focal points through the neuroaxis. About 20% of inheritable cases of ALS are due to mutation in the gene encoding the Cu/Zn superoxide dismutase (SOD1), causing the protein to misfold and form neurotoxic aggregates. Mutant SOD1 has been shown to impart its misfold onto natively folded wild-type SOD1 in living cells. Furthermore, misfolded wild-type SOD1 can itself induce further rounds of propagated SOD1 misfolding. Finally, this prion-like mechanism of propagated SOD1 misfolding can be transmitted from cell to cell in human cell culture. Here, we describe a protocol for the induction of wild-type SOD1 misfolding inside living cells and its subsequent transmission from cell to cell in a prion-like fashion. |
X Demographics
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Demographic breakdown
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Mendeley readers
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| Professor | 3 | 14% |
| Researcher | 2 | 10% |
| Student > Bachelor | 1 | 5% |
| Unspecified | 1 | 5% |
| Other | 2 | 10% |
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| Unspecified | 1 | 5% |
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