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. 2015 Mar 23;112(14):4489–4494. doi: 10.1073/pnas.1419228112

Fig. 1.

Fig. 1.

Human SOD1 and its involvement in the motor-neuron disease ALS. (A) Structure of the native hSOD1 homodimer showing the positions of β-strands 1–8 and loops IV and VII of the active site (PDB ID code 1HL5). (B) The pathology of transgenic mice expressing hSOD1 variants is mainly confined to the spinal cord, giving disease characteristics similar to those of human ALS. (C) Immunohistochemistry of spinal ventral horn in hSOD1G85R mouse using the 57–72 antibody showing typical accumulation of hSOD1 deposits in a motor neuron. (D) The amino acid sequence of the wild-type hSOD1 subunit, showing the positions of native secondary structure and the peptides used for generation of the antibodies used in this study.