Although the mechanisms and causal relationships have yet to be elucidated, it has been proposed that neuronal protein inclusions, such as those formed by mutated SOD1 or TDP43 (43-kDa transactivator response region (TAR) DNA-binding domain protein) which have been found in ALS and in frontotemporal dementia, could cause their toxicity by acting as surface attractants that sequester vital components of the transcriptional machinery, as was first proved for huntingtin (Cha, 2000). The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.