4364 N-Acetyl-L-aspartic acid N-Acetylaspartic acid is a derivative of aspartic acid. It is the second most concentrated molecule in the brain after the amino acid glutamate. It is synthesized in neurons from the amino acid aspartate and acetyl coenzyme A (acetyl CoA). The various functions served by N-acetylaspartic acid are still under investigation, but the primary proposed functions include (1) acting as a neuronal osmolyte that is involved in fluid balance in the brain, (2) serving as a source of acetate for lipid and myelin synthesis in oligodendrocytes (the glial cells that myelinate neuronal axons), (3) serving as a precursor for the synthesis of the important dipeptide neurotransmitter N-acetylaspartylglutamate (NAAG), and (4) playing a potential role in energy production from the amino acid glutamate in neuronal mitochondria. High neurotransmitter levels can lead to abnormal neural signaling, delayed or arrested intellectual development, and difficulties with general motor skills. When present in sufficiently high levels, N-acetylaspartic acid can be a neurotoxin, an acidogen, and a metabotoxin. A neurotoxin is a compound that disrupts or attacks neural tissue. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of N-acetylaspartic acid are associated with Canavan disease. N-acetylaspartic acid is an organic acid. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. Infants with acidosis have symptoms that include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart abnormalities, seizures, coma, and possibly death. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and flapping tremors. 997-55-7 65065 C6H9NO5 White powder. 137 - 140°C 675 mg/mL The primary proposed functions of N-acetylaspartic acid include: 1) A neuronal osmolyte that is involved in fluid balance in the brain; 2) a source of acetate for lipid and myelin synthesis in oligodendrocytes, the glial cells that myelinate neuronal axons; 3) a precursor for the synthesis of the important neuronal dipeptide N-acetylaspartylglutamate; 4) N-acetylaspartic acid may also be involved in energy production from the amino acid glutamate in neuronal mitochondria. Excess amounts of N-acetyl-L-aspartic acid accumulated in plasma, spinal fluid, and urine have been found to be toxic. On the other hand, decreased concentration of N-acetyl-L-aspartic acid according to the in vivo spectrum of the brain has been related to disorders such as hypoacetylaspartia. No indication of carcinogenicity to humans (not listed by IARC). This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation. Chronically high levels of N-acetyl-aspartate are associated with Canavan Disease. 2014-08-29T06:18:34Z 2018-03-21T17:46:17Z N-acetylaspartate C01042 21547 CPD-420 true [H][C@@](CC(O)=O)(N=C(C)O)C(O)=O C6H9NO5 InChI=1S/C6H9NO5/c1-3(8)7-4(6(11)12)2-5(9)10/h4H,2H2,1H3,(H,7,8)(H,9,10)(H,11,12)/t4-/m0/s1 InChIKey=OTCCIMWXFLJLIA-BYPYZUCNSA-N 175.1394 175.048072403 Endogenous Solid HMDB00812 CHEMBL1162494 58576 Montoro, Fernando; Calatayud, Jose; Vilar, Angel. N-Acetyl-L-aspartic acid. Span. (1983), 5 pp.