N-Acetyl-L-aspartic acid is an acetylated form of L-aspartic acid.1 It is formed from L-aspartate and acetyl-CoA by L-aspartate N-acetyltransferase (Asp-NAT) in neuronal mitochondria.2 N-Acetyl-L-aspartic acid is hydrolyzed to L-aspartate and acetate by aspartoacylase (ASPA), which is expressed by oligodendrocytes. N-Acetyl-L-aspartic acid levels are increased in the brain of patients with Canavan disease, an autosomal recessive disorder caused by loss-of-function ASPA mutations and characterized by cognitive and motor impairments.3,2,1
1.Baslow, M.H.A review of phylogenetic and metabolic relationships between the acylamino acids, N-acetyl-?-aspartic acid and N-acetyl-?-histidine, in the vertebrate nervous systemJ. Neurochem.68(4)1335-1344(1997) 2.Madhavarao, C.N., Arun, P., Moffett, J.R., et al.Defective N-acetylaspartate catabolism reduces brain acetate levels and myelin lipid synthesis in Canavan's diseaseProc. Natl. Acad. Sci. USA102(14)5221-5226(2004) 3.Bannerman, P., Guo, F., Chechneva, O., et al.Brain Nat8l knockdown suppresses spongiform leukodystrophy in an aspartoacylase-deficient Canavan disease mouse modelMol. Ther.26(3)793-800(2018)