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(PubMed) Namazi N, Larijani B, Azadbakht L

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Disorders of Glutamate/Glutamine and Aspartate/Asparagine Metabolism Aspartoacylase Deficiency Aspartoacylase (ASPA) deficiency, also known as Canavan disease, is caused by mutations in the aspartoacylase gene and leads to a high accumulation of N-acetylaspartate (NAA) and its derivative N-acetyl-aspartyl-glutamate (NAAG) in the brain [91]