产品名称
ACY2 Rabbit Polyclonal Antibody
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
https://www.uniprot.org/uniprot/443
Human Swissprot No.
P45381
Human Swissprot Link
https://www.uniprot.org/uniprotkb/P45381/entry
Mouse Gene Link
https://www.uniprot.org/uniprot/11484
Mouse Swissprot No.
Q8R3P0
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/Q8R3P0
Rat Gene Link
https://www.uniprot.org/uniprot/79251
Rat Swissprot Link
https://www.uniprot.org/uniprotkb/Q9R1T5
免疫原
Synthesized peptide derived from human ACY2 AA range: 67-117
特异性
This antibody detects endogenous levels of ACY2 at Human/Mouse/Rat
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
组织表达
Brain white matter, skeletal muscle, kidney, adrenal glands, lung and liver.
细胞定位
Cytoplasm. Nucleus .
功能
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.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.