A crucial study relating VEGF deficit with ALS was obtained by the design of the mutant mice VEGFδ/δ, in which the hypoxia response element located in the promoter region of the VEGF gene was deleted, causing a decrease in VEGF levels with time that leads to adult-onset motoneuron degeneration reminiscent of ALS (Oosthuyse et al., 2001). Here, VEGFA is linked to amyotrophic lateral sclerosis.