在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

Gba Antibody, FITC conjugated

  • 中文名稱:
    Gba兔多克隆抗體, FITC偶聯
  • 貨號:
    CSB-PA009289HC01MO
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Mus musculus (Mouse) Gba Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    Gba
  • 別名:
    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
  • 反應種屬:
    Mouse
  • 免疫原:
    Recombinant Mouse Glucosylceramidase protein (20-515AA)
  • 免疫原種屬:
    Mus musculus (Mouse)
  • 標記方式:
    FITC
  • 克隆類型:
    Polyclonal
  • 抗體亞型:
    IgG
  • 純化方式:
    >95%, Protein G purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 產品提供形式:
    Liquid
  • 儲存條件:
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期:
    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.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 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.
  • 基因功能參考文獻:
    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

    顯示更多

    收起更多

  • 亞細胞定位:
    Lysosome membrane; Peripheral membrane protein; Lumenal side.
  • 蛋白家族:
    Glycosyl hydrolase 30 family
  • 數據庫鏈接:


主站蜘蛛池模板: 免费人成视频在线播放视频 | 久久人人爽人人爽人人片av东京热| 国产精品综合色区在线观看| 国产久热精品无码激情| 午夜国产免费视频亚洲| 欧美成aⅴ人高清免费观看| 夜夜躁狠狠躁日日躁视频| 精精国产xxxx视频在线播放| 久久久久国产一区二区| 国产成人久久综合77777| 久久久精品人妻一区二区三区gv| 久久无码人妻一区二区三区午夜 | 精品久久久久久777米琪桃花| 18级成人毛片免费观看| 日本三级吃奶头添泬无码苍井空 | 少妇极品熟妇人妻200片| 无码专区一va亚洲v专区在线| 大香伊蕉国产av| 国产高潮又爽又刺激的视频| 69风韵老熟女口爆吞精| 亚洲精品第一区二区三区| 成人动漫综合网| 久久精品女人天堂av麻| 亚洲国产av无码一区二区三区| 国产在线视频一区二区三区98 | 国产9 9在线 | 中文| 亚洲国产成人久久综合人| 久久99热只有频精品8| 免费看成人aa片无码视频吃奶| 国产一区二区三区四区五区vm| 青乐娱精品视频一国产分类| 国产青榴视频在线观看| 无码中文字幕色专区| 亚洲精品1卡2卡三卡4卡乱码| 中文字幕无码热在线视频| 久久丫精品系列| 全黄h全肉边做边吃奶视频| 日韩卡1卡2 卡三卡免费| 国产高清国产精品国产专区| 日本丰满少妇裸体自慰| 女女同性av片在线观看免费|