| 别名 | ACY 2, ACY2, Aminoacylase 2, ASP, ASPA, Aspartoacylase |
| 基因名称 | ASPA |
| 蛋白名称 | ACY2 |
| 推荐应用 | |
| 反应物种 | Human,Mouse,Rat |
| 存储缓冲液 | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Human Gene Link | 443 |
| Human Swissprot No. | P45381 |
| Mouse Gene ID | 11484 |
| Mouse Swissprot No. | Q8R3P0 |
| Rat Gene ID | 79251 |
| Rat Swissprot No. | Q9R1T5 |
| 特异性 | endogenous |
| 稀释度 | WB 1:1000-1:4000;IF 1:50-1:200;ELISA 1:5000-1:20000;IP 1:50-1:200; |
| 参考分子量 | MW(Observed):33kD |
| 预测分子量 | MW(Calculated):36kD |
| 运输及保存条件 | -15°C to -25°C/1 year(Do not lower than -25°C) |
| 宿主 | Rabbit |
| 同种型 | IgG,Kappa |
| 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], |
| 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 | Protein A |
| Clonality | Monoclonal |
| 修饰类型 | Unmodified |
| 细胞定位 | Cytoplasm. Nucleus . |
| 组织表达 | Brain white matter, skeletal muscle, kidney, adrenal glands, lung and liver. |
| 研究领域 | >>Alanine, aspartate and glutamate metabolism;>>Histidine metabolism;>>Metabolic pathways |
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