The marked vacuolar changes of the white matter found in the l2hgdh-/- mice are reminiscent of Canavan disease but marginally involve the hippocampus and the cerebellum and not the spinal cord, in striking contrast to the spongy degeneration reported in the Nur7 aspartoacylase-deficient mouse model of Canavan disease [45]. The gene discussed is ASPA; the disease is Canavan disease.