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Disulfide-reduced ALS variants of Cu, Zn superoxide dismutase exhibit increased populations of unfolded species.
Metal-free ALS variants of dimeric human Cu,Zn-superoxide dismutase have enhanced populations of monomeric species.
Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis.
Amyotrophic Lateral Sclerosis
Folding of the RNA recognition motif (RRM) domains of the amyotrophic lateral sclerosis (ALS)-linked protein TDP-43 reveals an intermediate state.
Structural basis for mutation-induced destabilization of profilin 1 in ALS.
Characterization of TDP-43 RRM2 Partially Folded States and Their Significance to ALS Pathogenesis.
Structural Rearrangement upon Fragmentation of the Stability Core of the ALS-Linked Protein TDP-43.
Nonnative structure in a peptide model of the unfolded state of superoxide dismutase 1 (SOD1): Implications for ALS-linked aggregation.
Friction-Limited Folding of Disulfide-Reduced Monomeric SOD1.
Motor Neuron Disease