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Gephyrin Rabbit pAb (bs-6644R)  
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產品編號 bs-6644R
英文名稱 Gephyrin Rabbit pAb
中文名稱 橋尾蛋白抗體
別    名 Domain E; Domain G; GEPH; GEPH_HUMAN; GPH; GPHN; GPHRYN; Molybdopterin molybdenumtransferase; MPT adenylyltransferase; MPT Mo-transferase.  
研究領域 神經生物學  信號轉導  細胞粘附分子  細胞表面分子  細胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse (predicted: Human,Rat,Rabbit,Pig,Cow,Dog,Horse)
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 83 kDa
檢測分子量
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Gephyrin: 231-330/736 
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
產品介紹 The sub-membraneous region at the postsynaptic membrane contains a number of proteins critical for receptor targeting. Gephyrin is a microtubule-associated protein highly expressed in brain and localized to neuronal postsynaptic membranes. Gephyrin is essential for the postsynaptic localization of the inhibitory glycine receptor and is thought to anchor the receptor to subsynaptic microtubules. The protein is expressed in most mammalian tissues with predominant expression in brain. At least five additional splice variants of Gephyrin ranging in molecular weight have been identified in rat and human brain tissue.

Function:
Microtubule-associated protein involved in membrane protein-cytoskeleton interactions. It is thought to anchor the inhibitory glycine receptor (GLYR) to subsynaptic microtubules (By similarity). Catalyzes two steps in the biosynthesis of the molybdenum cofactor. In the first step, molybdopterin is adenylated. Subsequently, molybdate is inserted into adenylated molybdopterin and AMP is released.

Subunit:
Homotrimer. Interacts with GABARAP (By similarity).

Subcellular Location:
Cell junction, synapse (By similarity). Cell junction, synapse, postsynaptic cell membrane; Peripheral membrane protein; Cytoplasmic side (By similarity). Cytoplasm, cytoskeleton (By similarity). Note=Cytoplasmic face of glycinergic postsynaptic membranes (By similarity).

DISEASE:
Defects in GPHN are the cause of molybdenum cofactor deficiency type C (MOCOD type C) [MIM:252150]. MOCOD type C is an autosomal recessive disease which leads to the pleiotropic loss of all molybdoenzyme activities and is characterized by severe neurological damage, neonatal seizures and early childhood death.

Similarity:
In the N-terminal section; belongs to the moaB/mog family.
In the C-terminal section; belongs to the moeA family.

SWISS:
Q9NQX3

Gene ID:
10243

Database links:

Entrez Gene: 10243 Human

Entrez Gene: 268566 Mouse

Entrez Gene: 64845 Rat

Omim: 603930 Human

SwissProt: Q9NQX3 Human

SwissProt: Q8BUV3 Mouse

SwissProt: Q03555 Rat

Unigene: 208765 Human

Unigene: 341742 Mouse

Unigene: 363753 Mouse

Unigene: 453131 Mouse

Unigene: 11032 Rat



產品圖片
Sample: Lung (Mouse) Lysate at 40 ug Primary: Anti- Gephyrin (bs-6644R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 83 kD Observed band size: 83 kD
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