ALS Compendium

Title: An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity

Year Published: 2011

Observation: A315T mutant TDP43 forms protein aggregates and aberrant TDP43 species. TDP43 A315T flanting sequences resemble prions and form beta sheets. TDP43 peptides form amyloid fibrils

Comment: Show biochemical similarities between TDP-43 and prion proteins

Citation: Guo, W., Chen, Y., Zhou, X., Kar, A., Ray, P., Chen, X., … Wu, J. Y. (2011). An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity. Nature Structural & Molecular Biology, 18(7), 822–30. doi:10.1038/nsmb.2053

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