Table 1.

Comparison of Measured (Z_CE) and Predicted (Z_seq) Values of Net Charge of WT and ALS-variant D90A, G85R, G93R, and E100K SOD1 at pH 7.4, 20°C

	Net charge (ZCE and Zseq)a
SOD1b	WTc	D90A	G85R	G93R	E100K
apo(a) ZCE	−12.13 ± 0.08	−9.30 ± 0.02	−8.71 ± 0.01	−8.91 ± 0.04	−8.36 ± 0.02
Zseq	−13.0	−11.0	−11.0	−11.0	−9.0
apo(a1) ZCE	N/A	N/A	N/A	−5.04 ± 0.02	N/A
Zseq	−13.0	−11.0	−11.0	−11.0	−9.0
Zn1(b) ZCE	−8.12 ± 0.23	−6.12 ± 0.01	−6.38 ± 0.01	−6.25 ± 0.02	−5.42 ± 0.01
Zseq	−10.3	−8.3	−8.3	−8.3	−6.3
Zn1(c) ZCE	N/A	−7.22 ± 0.03	N/A	N/A	N/A
Zseq	−10.3	−8.3	−8.3	−8.3	−6.3
Zn2(e) ZCE	−8.62 ± 0.30	−6.46 ± 0.08	−6.45 ± 0.10	−6.09 ± 0.01	−5.81 ± 0.11
Zseq	−7.6	−5.6	−5.6	−5.6	−3.6
Zn2(f) ZCE	−8.45 ± 0.27	−6.23 ± 0.10	N/A	−6.08 ± 0.03	−5.85 ± 0.07
Zseq	−7.6	−5.6	−5.6	−5.6	−3.6
Zn3(j) ZCE	N/A	N/A	N/A	N/A	−5.48 ± 0.01
Zseq	−7.9	−5.9	−5.9	−5.9	−3.9
Zn3(i) ZCE	−8.55 ± 0.11	−6.02 ± 0.02	N/A	−6.20 ± 0.01	−5.83 ± 0.01
Zseq	−7.9	−5.9	−5.9	−5.9	−3.9
Zn4(m) ZCE	N/A	N/A	N/A	N/A	−5.44 ± 0.01
Zseq	−8.2	−6.2	−6.2	−6.2	−4.2
Zn4(k) ZCE	−8.54 ± 0.12	−6.12 ± 0.01	N/A	−6.14 ± 0.01	−5.72 ± 0.03
Zseq	−8.2	−6.2	−6.2	−6.2	−4.2

^aAll values of net charge are listed per SOD1 dimer.

^bThe labels “a”, “a₁”, “b”, etc., correspond to labels of electrophoretically unique states in Figure 2 of main text and are discussed in Supporting Information.

^cValues for WT SOD1 are from a previous study.²¹