Price range: $268.00 through $328.00
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],
| Size | 50μL/100μL |
| Target | ASPA |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, IHC, IF, IP, ELISA |
| MW(Calculated) | 36kD |
| MW(Observed) | 33kD |
| Host Species | Rabbit |
| Isotype | IgG,Kappa |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | IHC 1:200-1:1000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; IP 1:50-1:200; / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004) |
| Formulation | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Purification | Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | |
| Clonality | Monoclonal |
| Clone Number | RM1339R |
| Immunogen | The specific immunogen used to produce this antibody is proprietary information. |
| Specificity | Endogenous |
| Gene Name | ASPA ACY2 ASP |
| Protein Name | ACY2 |
| Other Name | |
| Species | Human |
| Gene ID | 443 |
| Uniprot | P45381, |
| Species.1 | Mouse |
| Gene ID.1 | 11484 |
| Uniprot.1 | Q8R3P0, |
| Species.2 | Rat |
| Gene ID.2 | 79251 |
| Uniprot.2 | Q9R1T5 |
| Cellular Localization | Cytoplasm. Nucleus . |
| Tissue Expression | Brain white matter, skeletal muscle, kidney, adrenal glands, lung and liver. |
| Signaling_pathway | |
| Research Areas | >>Alanine, aspartate and glutamate metabolism; / >>Histidine metabolism; / >>Metabolic pathways |
| 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 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., |
