The discovery in the past decade that transgenic mice with a homozygous deletion in the hypoxia response element site in the VEGF promoter (VEGFδ/δ mice) expressed reduced levels of VEGF (25–40% less) and developed late-onset motoneuron pathology reminiscent of amyotrophic lateral sclerosis (ALS), opened a new field of research on this dramatic disease. This evidence concerns the gene VEGFD and amyotrophic lateral sclerosis.