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

SELENON Antibody

  • 中文名稱:
    SELENON兔多克隆抗體
  • 貨號:
    CSB-PA878943LA01HU
  • 規格:
    ¥440
  • 圖片:
    • Western Blot
      Positive WB detected in: Rat brain tissue, Rat lung tissue
      All lanes: SELENON antibody at 4.2μg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 66, 63 kDa
      Observed band size: 66 kDa
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) SELENON Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    SELENON
  • 別名:
    CFTD antibody; MDRS1 antibody; RSMD1 antibody; RSS antibody; Selenoprotein N antibody; Selenoprotein N, 1 antibody; SelN antibody; SELN_HUMAN antibody; sepn1 antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human, Rat
  • 免疫原:
    Recombinant Human Selenoprotein N protein (135-219AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Non-conjugated

    本頁面中的產品,SELENON Antibody (CSB-PA878943LA01HU),的標記方式是Non-conjugated。對于SELENON Antibody,我們還提供其他標記。見下表:

    可提供標記
    標記方式 貨號 產品名稱 應用
    HRP CSB-PA878943LB01HU SELENON Antibody, HRP conjugated ELISA
    FITC CSB-PA878943LC01HU SELENON Antibody, FITC conjugated
    Biotin CSB-PA878943LD01HU SELENON Antibody, Biotin conjugated ELISA
  • 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
  • 應用范圍:
    ELISA, WB
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:500-1:5000
  • Protocols:
  • 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:
    Plays an important role in cell protection against oxidative stress and in the regulation of redox-related calcium homeostasis. Regulates the calcium level of the ER by protecting the calcium pump ATP2A2 against the oxidoreductase ERO1A-mediated oxidative damage. Within the ER, ERO1A activity increases the concentration of H(2)O(2), which attacks the luminal thiols in ATP2A2 and thus leads to cysteinyl sulfenic acid formation (-SOH) and SEPN1 reduces the SOH back to free thiol (-SH), thus restoring ATP2A2 activity. Acts as a modulator of ryanodine receptor (RyR) activity: protects RyR from oxidation due to increased oxidative stress, or directly controls the RyR redox state, regulating the RyR-mediated calcium mobilization required for normal muscle development and differentiation.; Essential for muscle regeneration and satellite cell maintenance in skeletal muscle.
  • 基因功能參考文獻:
    1. Case Report: rigid spine muscular dystrophy 1 in a compound heterozygote with two novel mutations in SEPN1 gene; a novel missense mutation (c.1384T>C; p.Sec462Arg) and a novel nonsense mutation (c.1525C>T; p.Gln509Ter), inherited from his father and mother respectively. PMID: 27863379
    2. We report two previously undescribed mutations in SEPN1. Our study adds two novel homozygous mutations to the number of reported pathogenic SEPN1 variants. PMID: 26780752
    3. The physiological function of SelN in muscle tissue and the pathogenesis leading to SEPN1-related myopathies. [Review] PMID: 22527882
    4. Data show that the spectrum of severity of SEPN1-related myopathiesis wider than previously reported. PMID: 21670436
    5. Data show that Argonaute 2 expression is critical for stem cells to escape senescence by downregulating miR10b and miR23b, and that selenoprotein N1 is also involved in ATSC survival and self-renewal through ROS-mediated p38 MAPK inactivation. PMID: 21241449
    6. this series of patients illustrates the clinical, histopathological and MRI findings of SEPN1-related myopathy. It also adds new mutations to the limited number of fully described pathogenic SEPN1 variants. PMID: 20937510
    7. Mutations of the selenoprotein N gene, which is implicated in rigid spine muscular dystrophy, cause the classical phenotype of multiminicore disease PMID: 12192640
    8. A new SEPN1 point mutation, 943g->A causing G315S was found in a rigid spine muscular dystrophy patient with cor pulmonale. PMID: 15668457
    9. SEPN1 mutation analysis revealed that the patient was a compound heterozygote: a previously described insertion (713-714 insA), and a novel nonsense mutation (R439stop). PMID: 15792869
    10. Two patients with 'Dropped head syndrome' due to mutations in SEPN1 genes. PMID: 15961312
    11. SEPN1 is the second genetic cause of CFTD and the first cause of autosomal recessive CFTD to be identified to our knowledge. CFTD is the fourth clinicopathological presentation that can be associated with mutations in SEPN1. PMID: 16365872
    12. identification of this mutation affecting a conserved base in the selenocysteine insertion sequence functional motif thereby reveals the structural basis for a novel pathological mechanism leading to SEPN1-related myopathy PMID: 16498447
    13. We report on the possible molecular mechanism behind these mutations in SEPN1. Our study clarifies molecular mechanisms of this muscular disorder. PMID: 16779558
    14. SEPN1 and RYR1 are required for the same cellular differentiation events and are needed for normal calcium fluxes PMID: 18713863
    15. Data highlights the importance of the SRE element during SelN expression and illustrates a novel molecular mechanism by which point mutations may lead to SEPN1-related myopathy. PMID: 19067361
    16. SelN plays a key role in redox homeostasis and human cell protection against oxidative stress. PMID: 19557870
    17. The Alu-derived exon 3 of human SEPN1 acquired its muscle-specific splicing activity after the divergence of humans and chimpanzees, suggesting its potential role in human evolution. PMID: 18841251

    顯示更多

    收起更多

  • 相關疾病:
    Rigid spine muscular dystrophy 1 (RSMD1); Myopathy, congenital, with fiber-type disproportion (CFTD)
  • 亞細胞定位:
    [Isoform 2]: Endoplasmic reticulum membrane.
  • 組織特異性:
    Isoform 1 and isoform 2 are expressed in skeletal muscle, brain, lung and placenta. Isoform 2 is also expressed in heart, diaphragm and stomach.
  • 數據庫鏈接:

    HGNC: 15999

    OMIM: 255310

    KEGG: hsa:57190

    STRING: 9606.ENSP00000355141

    UniGene: Hs.323396



主站蜘蛛池模板: 日本三级在线观看免费| 国产精品久久久久久不卡盗摄 | 亚洲成本人无码薄码区| 欲女熟妇国产一区二区| 人妻中出受孕 中文字幕在线| 亚洲国产精品无码久久久久高潮| 乱人伦人妻中文字幕无码| 中文字幕无码不卡免费视频 | 久久久久人妻一区二区三区| 欧美性大战xxxxx久久久| 亚洲国产综合精品 在线 一区| 免费无码又爽又刺激高潮软件| 中文字幕精品视频在线看免费| 亚洲精品中文字幕乱码4区| 特黄特色大片免费播放器图片| 午夜家庭影院| 中文字字幕在线中文乱码| 亚洲色欲色欲天天天www| 中文字字幕在线成人av电影| 动漫av纯肉无码av在线播放 | 国产1卡2卡三卡四卡精品| 国产精品爽爽久久久久久蜜臀| 久久久国产精华液| 高潮内射双龙视频| 国产一区二区三精品久久久无广告 | 偷国产乱人伦偷精品视频 | 精品亚洲成a人在线观看| 欧美人与动欧交视频| 亚洲高请码在线精品av| 亚洲精品久久久久久动漫| av无码精品一区二区三区宅噜噜| 国产a精彩视频精品视频下载| 人妻丰满熟妇a无码区| 久久99精品国产麻豆婷婷洗澡 | 激情综合色五月六月婷婷 | 国产精品无码一区二区在线看| 国产精品欧美亚洲韩国日本久久| 亚洲www啪成人一区二区| 国产饥渴孕妇在线播放| 性色av无码不卡中文字幕| 久久久久国产一区二区|