Deposition of complement proteins, particularly C1q and C3, has been identified at the motor end plates of the neuromuscular junction (NMJ) in both sporadic ALS patients and SOD1-ALS mice, and has been associated with the denervation of muscle endplates.57,58 We detected increased C1q and C3 deposition on the sarcolemma, in part co-localized with the NMJs in skeletal muscle biopsies of two patients with different genetic forms of CMT (FIG4, GDAP1). The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.