EC Number   |
Natural Substrates |
|---|
   3.5.1.15 | N-acetyl-L-aspartate + H2O |
- |
| 3.5.1.15 | N-acetyl-L-aspartic acid + H2O |
- |
| 3.5.1.15 | N-acetylaspartate + H2O |
- |
| 3.5.1.15 | N-acetylaspartic acid + H2O |
- |
| 3.5.1.15 | N-acetyl-L-aspartate + H2O |
aspartoacylase is a key enzyme in the human central nervous system. N-Acetyl-L-aspartate is a precursor for the synthesis of the dipeptide N-acetylaspartyl-glutamate, which participates in the neuromodulation of metabotropic and NMDA receptors (NMDA is N-methyl-D-aspartate), regulates the intracellular pressure in neurons and is involved in the energy generation from glutamate anions in neuronal mitochondria. N-Acetyl-L-aspartate is a source of acetyl groups for the construction of myelin sheath in the brain. Therefore, maintenance of the N-acetyl-L-aspartate level ensures proper development and functions of white matter |
| 3.5.1.15 | N-acetylaspartate + H2O |
aspartocylase deficiency results in elevated levels of substrate, brain edema and dysmyelination |
| 3.5.1.15 | N-acetyl-L-aspartate + H2O |
deficiency in enzyme activity leads to spongiform degeneration of the white matter of the brain and is the established cause of Canavan disease |
| 3.5.1.15 | N-acetylaspartic acid + H2O |
enzyme deficiency causes the Canavan disease, an autosomal-recessive neurodegenerative disorder |
| 3.5.1.15 | N-acetylaspartic acid + H2O |
enzyme deficiency, due to mutations of aspartoacylase II, causes the Canavan disease, which is associated with optical neuropathy |
| 3.5.1.15 | N-acetyl-L-aspartate + H2O |
enzyme mutations cause the Canavan disease |
