Target intelligence / Profile preview

Aspartoacylase

Molecular classification: Enzyme, Hydrolase

Overview

Aspartoacylase is a hydrolytic enzyme that catalyzes the deacylation of N-acetyl-L-aspartic acid (NAA) into aspartic acid and acetic acid. It is crucial for regulating NAA levels in the brain, which is essential for white matter integrity and myelination. Deficiency leads to Canavan disease, a fatal leukodystrophy.

Other names: Aminoacylase IIACY2N-acetyl-L-aspartate amidohydrolase

Mechanism of action

Hydrolyzes N-acetyl-L-aspartic acid into aspartate and acetate; Zinc-dependent hydrolase

Biological functions

Regulation of N-acetyl-L-aspartic acid levelsMyelinationAspartic acid productionAcetic acid production

Disease associations

Canavan diseaseLeukodystrophy

Safety considerations

Canavan disease (due to deficiency)Potential neurotoxicity from NAA accumulation

Biomarkers

N-acetyl-L-aspartic acid (NAA) levels

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