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

Recombinant Human Lipopolysaccharide-responsive and beige-like anchor protein (LRBA), partial

  • 中文名稱:
    人LRBA重組蛋白
  • 貨號:
    CSB-EP013070HU(N)
  • 規格:
    ¥1344
  • 圖片:
    • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
  • 其他:

產品詳情

  • 純度:
    Greater than 90% as determined by SDS-PAGE.
  • 基因名:
    LRBA
  • Uniprot No.:
  • 別名:
    Beige-like protein; BGL; C80285; CDC4-like protein; CDC4L; CVID8; D3Ertd775e; Lab300; LBA; Lipopolysaccharide-responsive and beige-like anchor protein; LPS-responsive vesicle trafficking beach and anchor containing; Lrba; LRBA_HUMAN; Vesicle trafficking beach and anchor containing
  • 種屬:
    Homo sapiens (Human)
  • 蛋白長度:
    Partial
  • 來源:
    E.coli
  • 分子量:
    31.6kDa
  • 表達區域:
    1-251aa
  • 氨基酸序列
    MASEDNRVPSPPPTGDDGGGGGREETPTEGGALSLKPGLPIRGIRMKFAVLTGLVEVGEVSNRDIVETVFNLLVGGQFDLEMNFIIQEGESINCMVDLLEKCDITCQAEVWSMFTAILKKSIRNLQVCTEVGLVEKVLGKIEKVDNMIADLLVDMLGVLASYNLTVRELKLFFSKLQGDKGRWPPHAGKLLSVLKHMPQKYGPDAFFNFPGKSAAAIALPPIAKWPYQNGFTFHTWLRMDPVNNINVDKDK
    Note: The complete sequence may include tag sequence, target protein sequence, linker sequence and extra sequence that is translated with the protein sequence for the purpose(s) of secretion, stability, solubility, etc.
    If the exact amino acid sequence of this recombinant protein is critical to your application, please explicitly request the full and complete sequence of this protein before ordering.
  • 蛋白標簽:
    N-terminal 6xHis-tagged
  • 產品提供形式:
    Liquid or Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 緩沖液:
    If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol.
    Note: If you have any special requirement for the glycerol content, please remark when you place the order.
    If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose.
  • 儲存條件:
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質期:
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 貨期:
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事項:
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet & COA:
    Please contact us to get it.

產品評價

靶點詳情

  • 功能:
    May be involved in coupling signal transduction and vesicle trafficking to enable polarized secretion and/or membrane deposition of immune effector molecules.
  • 基因功能參考文獻:
    1. LRBA is required for hair bundle maintenance in cochlear hair cells and for hearing. PMID: 28893864
    2. The present results suggest that LRBA SNPs are associated with CWP susceptibility in a Chinese population. PMID: 28953250
    3. As diabetes was the presenting feature in six of nine individuals, we recommend that testing for LRBA mutations is considered in all patients with newly diagnosed neonatal diabetes and in those with infancy-onset diabetes (<12 months), especially when a recessive inheritance is suspected or additional autoimmune features are present PMID: 28473463
    4. Assessing total CTLA-4 expression levels was found to be optimal when restricting analysis to the CD45RA(-)Foxp3(+) fraction. CTLA-4 induction following stimulation, and the use of lysosomal-blocking compounds, distinguished CTLA-4 from LRBA mutations PMID: 28159733
    5. Case Report: potential causative role of LRBA gene mutations in juvenile arthritis. PMID: 28134088
    6. Among 2 brothers homozygous for LPS responsive beige-like anchor protein (LRBA) mutation, one developed Evans syndrome and deceased at age 8.5, and his brother carried the same homozygous LRBA mutation with early-onset erosive polyarthritis. PMID: 27057999
    7. mutations result in various immunodeficiency phenotypes PMID: 26707784
    8. homozygous frame shift mutation results in refractory Celiac dsease PMID: 26686526
    9. diagnosis of LRBA deficiency was confirmed by a fluorescence-activated cell sorting-based immunoassay PMID: 26745254
    10. Variants of LRBA were associated with common variable immunodeficiency. PMID: 26122175
    11. A homozygous missense mutation in lipopolysaccharide-responsive and beige-like anchor gene is associated with inflammatory bowel disease. PMID: 25479458
    12. LRBA mutation was associated with an autoimmune lymphoproliferative syndrome-like disease characterized by splenomegaly and lymphadenopathy, cytopenia, elevated double negative T cells and raised serum Fas ligand levels. PMID: 25931386
    13. Patients with LRBA deficiency manifested a dramatic and sustained improvement in response to abatacept, a CTLA4 (cytotoxic T lymphocyte antigen-4)-immunoglobulin fusion drug. PMID: 26206937
    14. LRBA deficiency is a novel cause of immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome and Treg cell deficiency associated with metabolic dysfunction and increased apoptosis of Treg cells. PMID: 25468195
    15. A truncating mutation in LRBA, which abolished protein expression, was identified as the most likely candidate in a consanguineous family with chronic inflammatory bowel disease-like disorder and combined immunodeficiency. PMID: 22721650
    16. mutations in LRBA cause an immune deficiency characterized by defects in B cell activation and autophagy and by susceptibility to apoptosis, associated with a clinical phenotype of hypogammaglobulinemia and autoimmunity PMID: 22608502
    17. The crystal structure of the aPH-BEACH domains of LRBA were studied. PMID: 15554694

    顯示更多

    收起更多

  • 相關疾病:
    Immunodeficiency, common variable, 8, with autoimmunity (CVID8)
  • 亞細胞定位:
    Cell membrane; Single-pass membrane protein. Endoplasmic reticulum. Golgi apparatus, trans-Golgi network. Lysosome.
  • 組織特異性:
    Ubiquitous.
  • 數據庫鏈接:

    HGNC: 1742

    OMIM: 606453

    KEGG: hsa:987

    STRING: 9606.ENSP00000349629

    UniGene: Hs.480938



主站蜘蛛池模板: 国产∨亚洲v天堂无码久久久| 理论片87福利理论电影| 国产乱子经典视频在线观看| 国产福利姬喷水福利在线观看| 日本大肚子孕妇交xxx| 亚洲a∨无码男人的天堂| 亚洲中文字幕无码爆乳app| 久久精品国产9久久综合| 精品国产乱码久久久软件下载 | 国产猛烈高潮尖叫视频免费| 国产精品无码一区二区在线| 在线天堂资源www在线中文| 亚洲国产av导航第一福利网| 中文字幕精品久久久久人妻红杏1| 国产suv精品一区二区883| 国产色青青视频在线观看撒| 深爱婷婷国产在线精品av| 风韵少妇性饥渴推油按摩视频 | 午夜亚洲乱码伦小说区69堂| 亚洲精品成人av在线观看爽翻天| 欧美人妻精品一区二区三区| www国产精品内射老熟女| 国产三级韩国三级日产三级| 天天做日日做天天添天天欢公交车 | 免费看国产曰批40分钟| 欧洲亚洲1卡二卡三卡2021| 久久人人爽人人爽人人片ⅴ | 成人性生交片无码免费看| 亚洲成av人在线视猫咪| 猫咪免费人成网站在线观看| 大肉大捧一进一出好爽视色大师 | 国产裸体xxxx视频在线播放 | 精品国产自在现线电影| 国产熟妇另类久久久久| 亚欧无线一线二线三线区别| 国产欧美日韩视频一区二区三区 | 亚洲综合无码av一区二区三区| 99国产精品无码| 精品熟人妻一区二区三区四区不卡| 国产做a爱免费视频在线观看| 欧美午夜成人片在线观看|