Characterization of the Partially Folded States of TDP-43 RRM2 and Their Significance to ALS Pathogenesis

The human protein TDP-43 is a major component of the cellular aggregates found in amyotrophic lateral sclerosis and other neurodegenerative diseases. Insoluble cytoplasmic aggregates isolated from the brain of amyotrophic lateral sclerosis and fronto-temporal lobar degeneration patients contain ubiquitinated, hyperphosphorylated and N-terminally truncated TDP-43. Truncated fragments of TDP-43 identified from patient tissues contain part of the second RNA recognition motif (RRM2) and the disordered C-terminus, indicating that both domains can be involved in aggregation and toxicity.

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