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【課題】新規ヒト銅－亜鉛スーパーオキシドジスムターゼ（ＳＯＤ１タンパク質）およびミスフォールド／凝集形態のＳＯＤ１を認識するヒト抗体、それらのフラグメント、誘導体および変異体の提供。【解決手段】記憶Ｂ細胞由来ヒトモノクローナル抗ＳＯＤ１抗体またはそのＳＯＤ１結合フラグメントであって、ミスフォールドまたは凝集ＳＯＤ１に結合でき、且つ、生理的ＳＯＤ１二量体よりミスフォールド又は凝集ＳＯＤ１を優先的に認識し、該抗体又はそのＳＯＤ１結合フラグメントは、特定のアミノ酸配列を含みまたは該配列から成るＳＯＤ１エピトープに特異的に結合し、特定の配列中に示される相補性決定領域（ＣＤＲs）、或いはそのＣＤＲの一つ以上が１又は２個のアミノ酸の置換を含むものを、その可変領域内に含むＳＯＤ１結合フラグメント。【選択図】なし
