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

GBE1 Antibody

  • 中文名稱:
    GBE1兔多克隆抗體
  • 貨號:
    CSB-PA754361ESR2HU
  • 規格:
    ¥440
  • 圖片:
    • Western blot
      All lanes: GBE1 antibody at 3.13μg/ml + Mouse liver tissue
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      predicted band size 80 kDa
      Observed band size: 80 kDa
    • Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA754361ESR2HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human thyroid tissue using CSB-PA754361ESR2HU at dilution of 1:100
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) GBE1 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    GBE1
  • 別名:
    1 antibody; 1,4 alpha glucan branching enzyme antibody; 1,4-alpha-glucan branching enzyme 1 antibody; 4-alpha-glucan-branching enzyme antibody; amylo (1,4 to 1,6) transglucosidase antibody; amylo (1,4 to 1,6) transglycosylase antibody; Andersen disease antibody; APBD antibody; Brancher enzyme antibody; GBE 1 antibody; GBE antibody; GBE1 antibody; gGlucan (1,4 alpha ), branching enzyme 1 antibody; GLGB_HUMAN antibody; Glucan (1,4 alpha) branching enzyme antibody; Glycogen branching enzyme antibody; Glycogen storage disease type IV antibody; Glycogen-branching enzyme antibody; GSD4 antibody; OTTHUMP00000213788 antibody; OTTHUMP00000213833 antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human, Mouse
  • 免疫原:
    Recombinant Human 1,4-alpha-glucan-branching enzyme protein (1-300AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Non-conjugated
  • 克隆類型:
    Polyclonal
  • 抗體亞型:
    IgG
  • 純化方式:
    Antigen Affinity Purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA, WB, IHC
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:1000-1:5000
    IHC 1:20-1:200
  • 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:
    Required for normal glycogen accumulation. The alpha 1-6 branches of glycogen play an important role in increasing the solubility of the molecule.
  • 基因功能參考文獻:
    1. Case Report: novel heterozygous variant (c.760A>G; p.Thr254Ala) in exon 6 of the GBE1 gene resulting in glycogen storage disease type IV. PMID: 27107456
    2. The crystal structure of GBE1 in complex with oligosaccharides was determined, the structural and molecular bases of Adult Polyglucosan Body Disease-linked missense mutations was investigated. PMID: 26199317
    3. The presence of polyglucosan bodies in intramuscular nerve twigs by itself and is not an indication of APBD mutation. PMID: 26670585
    4. GBE1 mutation is found in manifesting heterozygous patients with adult polyglucosan body disease PMID: 25665141
    5. Case Reports: novel missense/deletion mutations in GBE1 in glycogen storage disease type IV. PMID: 20058079
    6. GBE1 mutations can cause an early adult-onset relapsing-remitting form of polyglucosan body disease distinct from adult polyglucosan body disease in several ways, including younger age at onset. PMID: 24248152
    7. Compound heterozygous mutations in GBE1 were identified as the cause of lethal multiple pterygium syndrome in a family. PMID: 23218673
    8. this is the first epidemiologic study of the mutation frequency of the adult polyglucosan body disease -associated GBE1 mutation c.1076A>C in a large Ashkenazi Jewish cohort. PMID: 22943850
    9. APBD with GBE deficiency is a clinically homogenous disorder that should be suspected in patients with adult onset leukodystrophy or spastic paraplegia with early onset of urinary symptoms and spinal atrophy. PMID: 23034915
    10. A review of the literature for glycogen storage disease type IV patients with characterized molecular defects and deficient enzyme activity reveals most GBE1 mutations to be missense mutations clustering in the catalytic enzyme domain. PMID: 22305237
    11. Case Report: report an as yet undefined and different phenotype of glycogen storage disease with diminished branching enzyme activity associated with multisystemic involvement. PMID: 18392749
    12. GYS1 regulation by HIF plays a central role in the hypoxic accumulation of glycogen, and hypoxia also upregulates the expression of UTP:glucose-1-phosphate urydylyltransferase (UGP2) and 1,4-alpha glucan branching enzyme (GBE1) PMID: 20300197
    13. Nine novel GBE1 mutations were identified, including nonsense, missense, deletion, insertion, and splice-junction mutations. Implications for protein structure and interactions were modeled. PMID: 15452297
    14. Mutations in the GBE1 gene, located on chromosome 3, have been identified in phenotypes of glycogenosis 4. PMID: 17915577
    15. brain white matter degeneration in APBD may result from tissue damage involving axons and myelin in GBE missense mutation PMID: 17994551
    16. A c.1558delC frame shift mutation in exon 12 and a c.1999C>T mutation in exon 14 of the GBE1 gene were observed in a neonate with glycogen storage disease type IV. PMID: 18289670

    顯示更多

    收起更多

  • 相關疾病:
    Glycogen storage disease 4 (GSD4); Polyglucosan body neuropathy, adult form (APBN)
  • 蛋白家族:
    Glycosyl hydrolase 13 family, GlgB subfamily
  • 數據庫鏈接:

    HGNC: 4180

    OMIM: 232500

    KEGG: hsa:2632

    STRING: 9606.ENSP00000410833

    UniGene: Hs.436062



主站蜘蛛池模板: 中文 在线 日韩 亚洲 欧美| 欧美孕妇变态重口另类| 亚洲欧美一区二区三区| 日韩人妻无码精品系列| 欧美黑人激情性久久| 久久久久人妻一区精品| 中文字幕亚洲欧美日韩在线不卡| 亚洲精品日韩一区二区电影| 国产99在线 | 免费| 国产精品va无码二区| 熟妇人妻中文字幕无码老熟妇| 熟妇好大好深好满好爽| 亚洲无线观看国产精品 | 久久国产精品萌白酱免费| 国内精品久久久久久无码| 久久久国产乱子伦精品| 福利cosplayh裸体の福利| 无码一区二区三区中文字幕| 欧美人与动人物姣配xxxx| 少妇精品视频无码专区| 久久99精品国产麻豆宅宅| 西西人体大胆4444www| 久久99国产精品二区| 狠狠色噜噜狠狠狠狠五月婷| 国内精品久久毛片一区二区| 国产午夜亚洲精品不卡| 国产精品青青青高清在线 | 激烈的性高湖波多野结衣| 51国产偷自视频区视频| 久久久精品中文字幕麻豆发布| 亚洲欧美日韩综合久久| 欧美 日本 国产 在线a∨观看| 亚洲一区二区三区四区五区六区| 强奷人妻日本中文字幕| 无码av免费永久免费永久专区| 亚洲国产精品成人影片久久| 国产在线视频一区二区三区| 特黄特色的大片观看免费视频 | a∨在线视频播放| 熟妇人妻中文字幕无码老熟妇| 国产精品久久久久不卡绿巨人|