Figure 9: Illustration showing the different intracellular states observed for some SOD1 fALS mutants.
From: In-cell NMR reveals potential precursor of toxic species from SOD1 fALS mutants
Left, a subset of SOD1 fALS mutants accumulates in the cells in an unstructured form (blue), which is unable to bind zinc (cyan) even when the latter is supplemented in excess. Right, coexpression of the copper chaperone hCCS (orange) and incubation with excess copper (dark orange) restore the correct maturation pathway, and Cu(I),Zn-SOD1SS is formed. In-cell NMR spectra of G93A SOD1 in the two states are shown as an example.
