Figure 2.

CCS-D2 stabilizes fALS SOD1 mutants and allows zinc binding. (a) ¹H-¹⁵N NMR spectra of lysates from cells expressing [U-¹⁵N]-labelled G93A SOD1 alone (green) and together with [U-¹⁵N]-labelled CCS-D2 (black). (b) ¹H-¹⁵N NMR spectra of lysates from cells expressing [U-¹⁵N]-labelled I113T SOD1 alone (magenta) and together with [U-¹⁵N]-labelled CCS-D2 (black). (c,d) Detailed views of characteristic SOD1 signals in the ¹H-¹⁵N NMR spectra of lysates containing [U-¹⁵N]-labelled WT SOD1 alone (black), and WT SOD1 (red) and mutants G93A, I113T, A4V (green, magenta and azure, respectively) in the complex with CCS-D2, showing that SOD1 mutants experience chemical shift changes similar to WT SOD1 upon complex formation. See also Supplementary Figure S3.