Of these proteins, cytoskeletal proteins including phosphorylated neurofilament heavy chain (pNFH) and tau have been shown to be elevated in the CSF of neurodegenerative diseases and proposed as biomarkers for ALS [12–15] and neurofilament aggregates have been observed in spinal cord motor neurons of ALS patients [16]. This evidence concerns the gene NEFH and amyotrophic lateral sclerosis.