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    Rabbit Anti-Glycogenin 1/BF594 Conjugated antibody (bs-13447R-BF594)
    訂購熱線:400-901-9800
    訂購郵箱:sales@bioss.com.cn
    訂購QQ:  400-901-9800
    技術支持:techsupport@bioss.com.cn
    說 明 書: 100ul  
    100ul/2980.00元
    大包裝/詢價
    產品編號 bs-13447R-BF594
    英文名稱 Rabbit Anti-Glycogenin 1/BF594 Conjugated antibody
    中文名稱 BF594標記的糖原蛋白1
    別    名 Glycogenin; Glycogenin-1; Glycogenin1; GLYG_HUMAN; GN-1; GN1; GYG 1; GYG; GYG1.  
    規格價格 100ul/2980元 購買        大包裝/詢價
    說 明 書 100ul  
    研究領域 腫瘤  細胞生物  信號轉導  糖蛋白  新陳代謝  
    抗體來源 Rabbit
    克隆類型 Polyclonal
    交叉反應
    產品應用 ICC=1:50-200 IF=1:50-200 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 39kDa
    性    狀 Lyophilized or Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human Glycogenin 1
    亞    型 IgG
    純化方法 affinity purified by Protein A
    儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
    保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
    產品介紹 background:
    Glycogen synthesis is initiated by the autoglucosylation of Glycogenin-1. Specifically, Glycogenin-1 glucosylates itself to begin the synthesis of glycogen in mammalian skeletal muscle. It acts as the primer to which further glucose monomers may be added. All of the Glycogenin-1 molecules contain at least one glucosyl residue before autoglucosylation begins. The first step of the glycogen synthesis occurs when a glucose molecule from UDP-glucose binds to the hydroxyl group of Tyr 194 on the Glycogenin-1 molecule. Using its glucosyltransferase activity, Glycogenin-1 adds more glucoses, each one coming from UDP-glucose. The glycosylation process reaches a plateau when five new glucose residues have been added, at which point glycogen synthase (GS) takes over and further elongates the chain. Glycogenin-1 remains covalently attached to the reducing end of the glycogen molecule.

    Function:
    Self-glucosylates, via an inter-subunit mechanism, to form an oligosaccharide primer that serves as substrate for glycogen synthase.

    Subunit:
    Homodimer tightly complexed to the 86 kDa catalytic subunit of glycogen synthase GYS1.

    Post-translational modifications:
    Self-glycosylated by the transfer of glucose residues from UDP-glucose to itself, forming an alpha-1,4-glycan of around 10 residues attached to Tyr-195. Phosphorylated.

    DISEASE:
    Defects in GYG1 are the cause of glycogen storage disease type 15 (GSD15) [MIM:613507]. It is a metabolic disorder resulting in muscle weakness, associated with the glycogen depletion in skeletal muscle, and cardiac arrhythmia, associated with the accumulation of abnormal storage material in the heart. The skeletal muscle shows a marked predominance of slow-twitch, oxidative muscle fibers and mitochondrial proliferation.

    Similarity:
    Belongs to the glycogenin family.

    Database links:

    Entrez Gene: 2992 Human

    Omim: 603942 Human

    SwissProt: P46976 Human

    Unigene: 477892 Human



    Important Note:
    This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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