ACY2 rabbit pAb

catalytic activity:N-acyl-L-aspartate + H(2)O = a carboxylate + L-aspartate.,cofactor:Binds 1 zinc ion per subunit.,disease:Defects in ASPA are the cause of Canavan disease (CAND) [MIM:271900]; also known as spongy degeneration of the brain. CAND is a rare neurodegenerative condition of infancy or childhood characterized by white matter vacuolization and demeylination that gives rise to a spongy appearance. The clinical features are onset in early infancy, atonia of neck muscles, hypotonia, hyperextension of legs and flexion of arms, blindness, severe mental defect, megalocephaly, and death by 18 months on the average.,function:Catalyzes the deacetylation of N-acetylaspartic acid (NAA) to produce acetate and L-aspartate. NAA occurs in high concentration in brain and its hydrolysis NAA plays a significant part in the maintenance of intact white matter. In other tissues it act as a scavenger of NAA from body fluids.,similarity:Belongs to the aspA/astE family. Aspartoacylase subfamily.,subunit:Homodimer.,tissue specificity:Brain white matter, skeletal muscle, kidney, adrenal glands, lung and liver.,