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Gba Antibody

  • 货号：
    CSB-PA009289HA01MO
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western Blot
      Positive WB detected in: Mouse liver tissue, Mouse spleen tissue, Mouse lung tissue
      All lanes: Gba antibody at 3.5µg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 58 kDa
      Observed band size: 58 kDa
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Mus musculus (Mouse) Gba Polyclonal antibody
  • Uniprot No.：
    P17439
  • 基因名：
    Gba
  • 别名：
    GbaLysosomal acid glucosylceramidase antibody; Lysosomal acid GCase antibody; EC 3.2.1.45 antibody; Acid beta-glucosidase antibody; Beta-glucocerebrosidase antibody; Cholesterol glucosyltransferase antibody; SGTase antibody; EC 2.4.1.- antibody; Cholesteryl-beta-glucosidase antibody; EC 3.2.1.104 antibody; D-glucosyl-N-acylsphingosine glucohydrolase antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Mouse
  • 免疫原：
    Recombinant Mouse Glucosylceramidase protein (20-515AA)
  • 免疫原种属：
    Mus musculus (Mouse)
  • 标记方式：
    Non-conjugated

    本页面中的产品，Gba Antibody (CSB-PA009289HA01MO)，的标记方式是Non-conjugated。对于Gba Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA009289HB01MO Gba Antibody, HRP conjugated ELISA
    FITC CSB-PA009289HC01MO Gba Antibody, FITC conjugated
    Biotin CSB-PA009289HD01MO Gba Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramide/GlcCer into free ceramide and glucose. Thereby, plays a central role in the degradation of complex lipids and the turnover of cellular membranes. Through the production of ceramides, participates in the PKC-activated salvage pathway of ceramide formation. Also plays a role in cholesterol metabolism. May either catalyze the glucosylation of cholesterol, through a transglucosylation reaction that transfers glucose from glucosylceramide to cholesterol. The short chain saturated C8:0-GlcCer and the mono-unsaturated C18:0-GlcCer being the most effective glucose donors for that transglucosylation reaction. Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl-beta-D-glucoside to ceramide. Finally, may also hydrolyze cholesteryl-beta-D-glucoside to produce D-glucose and cholesterol.
  • 基因功能参考文献：
    1. Thus, while the underlying mechanism is not clear, this model shows that gba deficiency impacts the age of onset and disease duration in aged SNCA(A53T) mice, providing a valuable resource to identify modifiers, pathways and possible moonlighting roles of glucocerebrosidase in Parkinson pathogenesis. PMID: 29173981
    2. The data support the contention that prolonged antagonism of glucosylceramide synthase (GCS)in the central nervous system (CNS)can affect alpha-synuclein processing and improve behavioral outcomes. Hence, inhibition of GCS represents a disease-modifying therapeutic strategy for GBA-related synucleinopathies and conceivably for certain forms of sporadic disease PMID: 28223512
    3. These results indicate that Gba1 deficiency enhances neuronal vulnerability to neurodegenerative processes triggered by increased alpha-synuclein expression. PMID: 28969384
    4. This study demonstrated that the gba1 deficiency mice showed gene regulation expression of the type I interferon. PMID: 27175482
    5. Rab7 accumulated in GCase deficient cells, supporting the notion that lysosomal recycling is impaired. Since recombinant GCase can reverse ALR impairment, we anticipate that strategies to restore GCase activity in the brains of both sporadic patients with PD and those with GBA1 mutations will improve autophagy lysosomal pathway, preventing the accumulation of a-synuclein and spread of pathology. PMID: 27378698
    6. heterozygosity for a Gaucher disease-associated mutation in glucocerebrosidase interferes with alpha-synuclein degradation and contributes to its accumulation PMID: 25351739
    7. Data indicate that ABC transporter A family member 12 knockout (Abca12(-/-)) epidermis had 5-fold more beta-glucocerebrosidase (GCase) protein, and a 5-fold increase in GCase activity. PMID: 24293640
    8. These results demonstrate, for the first time, a novel function of GBA1 as a beta-ChlGlc-synthesizing enzyme. PMID: 24211208
    9. Substrate compositional variation with tissue/region and Gba1 mutations in mouse models--implications for Gaucher disease. PMID: 23520473
    10. GBA1 and GBA2 activities had characteristic differences between the studied fibroblast, liver and brain samples. PMID: 22659419
    11. results not only point to a fundamental role for GBA in immune regulation but also suggest that GBA mutations in GD may cause widespread immune dysregulation through the accumulation of substrates PMID: 22665763
    12. This study suggested that several leads connecting GBA1 mutations with alpha-synuclein misprocessing have emerged as potential targets for the treatment of GBA1-related synucleinopathies. PMID: 22327140
    13. IFG stabilizes GCase in tissues and serum and can reduce visceral substrates in vivo. PMID: 22167193
    14. Mutations in GBA1 can cause Parkinson disease-like alpha-synuclein pathology; rescuing brain glucocerebrosidase activity might represent a therapeutic strategy for GBA1-associated synucleinopathies. PMID: 21730160
    15. evidence for the involvement of deletion of the GBA1 gene in multiple cell lineages in nonneuronopathic type 1 Gaucher disease PMID: 20962279
    16. The saposin C deficient mice backcrossed to point mutated GCase mimics the central nervous system phenotype and biochemistry of some type 3 (neuronopathic) variants of Gaucher disease. PMID: 20047948
    17. isofagomine increases the activity of the Gaucher disease L444P mutant form of beta-glucosidase PMID: 20148966
    18. Saposin C has multiple roles in glycosphingolipid catabolism and functions in Central Nervous System independent of its role as an stabilizer of GCase. PMID: 20015957
    19. mRNA shows generalized low level expression early in gestation with gradual appearance of differential expression appearing around gestational age E14 and significantly increasing at term and into adulthood. PMID: 11749048
    20. Results indicate that glucocerebrosidase deficiency, even in the absence of large amounts of sphingolipid storage, can trigger an inflammatory reaction. PMID: 11994410
    21. data indicate that saposin C is required for acid beta-glucosidase resistance to proteolytic degradation in the cell PMID: 12813057

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  • 亚细胞定位：
    Lysosome membrane; Peripheral membrane protein; Lumenal side.
  • 蛋白家族：
    Glycosyl hydrolase 30 family
  • 数据库链接：

    KEGG: mmu:14466

    STRING: 10090.ENSMUSP00000076589

    UniGene: Mm.5031