"N-acetylaspartate, or NAA, is the acetylated form of the amino acid aspartate, and it is present exclusively in the nervous system. Indeed, NAA is one of the most highly concentrated chemicals found in the brain of humans and animals, and yet the functions served by this brain-specific metabolite remain elusive, and controversial. Despite the uncertainties surrounding the functions of NAA in the development and operation of the nervous system, this molecule has attracted the attention of researchers and clinicians for two distinct reasons.

A Brief Overview of N-Acetylaspartate and N-Acetylaspartylglutamate.- Expression of N-Acetylaspartate and N-Acetylaspartylglutamate in the Nervous System.- N-Acetylaspartate Metabolism in Neural Cells.- Naa Synthesis and Functional Roles.- Identity of the High-Affinity Sodium/Carboxylate Cotransporter NaC3 as the N-Acetyl-L-Aspartate Transporter.- Canavan Disease: Studies on the Knockout Mouse.- Functions Of N-Acetylaspartate and N-Acetylaspartylglutamate in Brain.- Control of Brain Volume during Hypoosmolality and Hyperosmolality.- Physiological Role of N-Acetylaspartate.- Defective Myelin Lipid Synthesis as a Pathogenic Mechanism of Canavan Disease.- Mutation Analysis of the Aspartoacylase Gene in Non-Jewish Patients with Canavan Disease.- Does ASPA Gene Mutation in Canavan Disease Alter Oligodendrocyte Development?.- Quantitation of NAA in the Brain by Magnetic Resonance Spectroscopy.- N-Acetyl-L-Aspartate in Multiple Sclerosis.- NAA and Higher Cognitive Function in Humans.- In Vivo NMR Measures of NAA and the Neurobiology of Schizophrenia.- N-Acetylaspartate as a Marker of Neuronal Injury in Neurodegenerative Disease.- Regulation of NAA-Synthesis in the Human Brain in Vivo: Canavan's Disease, Alzheimer's Disease and Schizophrenia.- Magnetic Resonance Spectroscopy for Monitoring Neuronal Integrity in Amyotrophic Lateral Sclerosis.- Hypoacetylaspartia: Clinical and Biochemical Follow-Up of a Patient.- Cellular Localization of NAAG.- Synthesis of N-Acetylaspartylglutamate (NAAG) and N-Acetylaspartate (NAA) in Axons and Glia of the Crayfish Medial Giant Nerve Fiber.- NAAG as a Neurotransmitter.- Glutamate Carboxypeptidase II (NAALADase) Inhibition as a Novel Therapeutic Strategy.- N-Acetylaspartylglutamate (NAAG) in Spinal Cord Injury and Disease.-N-Acetylaspartylglutamate (NAAG) in Pelizaeusmerzbacher Disease.- Concluding Remarks.