Immunogen
Synthesized peptide derived from human ACY2 AA range: 67-117
Specificity
This antibody detects endogenous levels of ACY2 at Human/Mouse/Rat
Source
Polyclonal, Rabbit,IgG
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB 1:500-2000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
Background
This gene encodes an enzyme that catalyzes the conversion of N-acetyl_L-aspartic acid (NAA) to aspartate and acetate. NAA is abundant in the brain where hydrolysis by aspartoacylase is thought to help maintain white matter. This protein is an NAA scavenger in other tissues. Mutations in this gene cause Canavan disease. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jul 2008],
Function
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 scaveng
Fields
>>Alanine, aspartate and glutamate metabolism;>>Histidine metabolism;>>Metabolic pathways
Human protein sequence Database
P45381
Mouse protein sequence database
Q8R3P0
Rat protein sequence database
Q9R1T5
Cellular localization
Cytoplasm. Nucleus .
Tissue expression
Brain white matter, skeletal muscle, kidney, adrenal glands, lung and liver.