PROBLEM TO BE SOLVED: To provide a novel human copper-zinc superoxide dismutase (SOD1 protein) and a human antibody that recognizes the misfolded/aggregated forms of SOD1 as well as fragments, derivatives and variants thereof.SOLUTION: The present invention provides a human monoclonal anti-SOD1 antibody derived from a human memory B cell or a SOD1-binding fragment thereof, and the antibody can bind to misfolded or aggregated SOD1 and more dominantly recognizes the misfolded or aggregated SOD1 than a physiological SOD1 dimer. The antibody or the SOD1-binding fragment thereof specifically binds to a SOD1 epitope comprising a specific amino acid sequence or consisting of the sequence and comprises in its variable region complementarity determining regions (CDRs) represented in the specific sequence or those including one or two substituted amino acids in one or more of the CDRs.SELECTED DRAWING: None
HUMAN ANTI-SOD1 ANTIBODY