Genetic linkage analysis first identified a missense point mutation in VAPB as the cause of a familial human motor neurone disease amyotrophic lateral sclerosis Type 8 (ALS8).1 The first cases were reported in Brazil, with others found subsequently in China, Germany, North America and Japan.2-5 The originally identified VAPBP56S mutation is the most frequent and has occurred independently on at least two occasions. Here, VAPB is linked to amyotrophic lateral sclerosis.