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Rabbit Anti-Glycogenin 1  antibody (bs-13447R)  
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產(chǎn)品編號 bs-13447R
英文名稱 Rabbit Anti-Glycogenin 1  antibody
中文名稱 糖原蛋白1
別    名 Glycogenin; Glycogenin-1; Glycogenin1; GLYG_HUMAN; GN-1; GN1; GYG 1; GYG; GYG1.  
研究領域 腫瘤  細胞生物  信號轉(zhuǎn)導  糖蛋白  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human,Mouse,Rat,Sheep,Cow,Dog)
產(chǎn)品應用 ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 39kDa
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Glycogenin 1: 251-350/350 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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.

SWISS:
P46976

Gene ID:
2992

Database links:

Entrez Gene: 2992 Human

Omim: 603942 Human

SwissProt: P46976 Human

Unigene: 477892 Human



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