| Primary Identifier | IPR016708 | Type | Family |
| Short Name | Aspartoacylase |
| description | Aspartoacylase (ASPA) is the N-acetylaspartate- (NAA)-hydrolysing enzyme. It catalyses the deacetylation of N-acetyl-L-aspartate to produce L-aspartate and acetate. N-phosphonomethyl-L-aspartate is a potent inhibitor of this enzyme []. There is a high concentration of (NAA) in neurons of the central nervous system, which is used as an indicator of neuronal viability. The myelin-associated pathology; Canavan disease (also known as spongy degeneration of the brain), is an inherited childhood disorder that results from a mutation of ASPA. The dysmyelination that results from the inactivation of ASPA is characterised by selective decreases as well as some increases in specific lipids []. |
