Concomitantly, we also observed significantly higher levels of NLRP3 in blood-derived macrophages derived from C9-positive subjects (Fig. 9, B and C) and the increased localization of p65 (one of the five components that form the NF-κB transcription family) in IBA1+ cells in postmortem spinal cord tissue from C9-ALS cases (fig. Here, C9 is linked to amyotrophic lateral sclerosis.