Thus, our results i) demonstrate the early involvement of NfH in pathology in SOD1 mice, ii) reveal the true abundance of NfH in the plasma of these mice and iii) suggest that an increase in the ratio of hyperphosphorylated NfH: variably phosphorylated NfH is indicative of pathology, at least in the SOD1G93A mouse model of ALS. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.