ASPA Rabbit mAb

活动价:

¥880.00

市场价:¥1280.00

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产品规格:
  • 50μL
货号:

FAB2621885

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商品描述
  • 靶点-Target

    ASPA

  • 反应性

    Human,Mouse,Rat

  • 应用

    WB,IHC,IF,IP,ELISA

  • 宿主物种-Host Species

    Rabbit

  • 同种型-Isotype

    IgG,Kappa

  • MW(Calculated)

    36kD

  • MW(Observed)

    33kD

  • 推荐稀释比

    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;

  • 组成

    PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA

  • 纯化工艺

    Protein A

  • 储存

    -15°C to -25°C/1 year(Do not lower than -25°C)

  • 克隆性

    Monoclonal

  • 克隆号

    PT1339R

  • 特异性

    Endogenous

  • 基因名称

    ASPA ACY2 ASP

  • 蛋白名称

    ACY2

  • Organism-1

    Human

  • 基因ID-1

    443

  • SwissProt-1

    P45381

  • Organism-2

    Mouse

  • 基因ID-2

    11484

  • SwissProt-2

    Q8R3P0

  • Organism-3

    Rat

  • 基因ID-3

    79251

  • SwissProt-3

    Q9R1T5

  • 背景

    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

    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.,