Figure 7: In the presence of Cu(I)-hCCS, all SOD1 mutants reach the folded mature state.
From: In-cell NMR reveals potential precursor of toxic species from SOD1 fALS mutants
Histidine regions of 1H NMR spectra acquired on human cells coexpressing unlabelled fALS-linked SOD1 mutants together with hCCS, in excess of both zinc and copper. The presence of Cu(I)-hCCS in the cytoplasm allows the formation of mature SOD1 (Cu(I),Zn-SOD1SS) for all mutants, with the exception of G85R. A small amount of E,Zn-SOD1 is still present for some mutants. Histidine protons unambiguously assigned to E,Zn-SOD1 and Cu(I),Zn-SOD1 species are indicated in light grey and dark grey, respectively.
