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信號轉(zhuǎn)導分子SCDO3抗體規(guī)格 英文名稱  Anti-SCDO3/Lunatic Fringe

中文名稱  信號轉(zhuǎn)導分子SCDO3抗體規(guī)格 

別    名 3-N-acetylglucosaminyltransferase lunatic fringe; Beta-1; Beta-13-N-acetylglucosaminyltransferase lunatic fringe; lfng; LFNG_HUMAN; O-fucosylpeptide 3-beta-N-acetylglucosaminyltransferase; SCDO3.

產(chǎn)品屬性：

濃    度  1mg/1ml

規(guī) 格  0.2ml/200μg

抗體來源  Rabbit

克隆類型   polyclonal

交叉反應   Human, Mouse, Rat, Chicken, Pig, Cow, Horse, Rabbit, Sheep

產(chǎn)品類型   一抗  

研究領域    細胞生物 發(fā)育生物學 神經(jīng)生物學 信號轉(zhuǎn)導

蛋白分子量  predicted molecular weight: 42kDa 

性    狀   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human SCDO3/Lunatic Fringe

亞    型  IgG

純化方法   affinity purified by Protein A

儲 存 液   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

信號轉(zhuǎn)導分子SCDO3抗體規(guī)格 產(chǎn)品應用   WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500

（石蠟切片需做抗原修復） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

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. 

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

產(chǎn)品介紹 Three mammalian fringe family members, namely Manic Fringe, Radical Fringe and Lunatic Fringe, have been identified as proteins related to Drosophila fringe, a protein involved in development. Fringe proteins act upstream of the Notch signaling pathway and are involved in boundary determination during segmentation. Each mammalian fringe displays different patterns of expression, though all are expressed in the mouse embryo as well as in many adult tissues. Lunatic Fringe, also known as LFNG or SCDO3, is a 379 amino acid single-pass type II membrane protein that localizes to the membrane of the Golgi apparatus. Functioning as a glycosyltransferase, Lunatic Fringe acts a critical mediator of somite patterning and segmentation and plays a fundamental role in initiating the elongation of O-linked fucose residues that are attached to Notch molecules. Defects in the gene encoding Lunatic Fringe are the cause of autosomal recessive spondylocostal dysostosis 3 (SCDO3), a disorder that arises during embryonic development and is characterized by rib anomalies and multiple vertebrate segmentation. Three isoforms of Lunatic Fringe are expressed due to alternative splicing events.

Function : Glycosyltransferase that initiates the elongation of O-linked fucose residues attached to EGF-like repeats in the extracellular domain of Notch molecules. Decreases the binding of JAGGED1 to NOTCH2 but not that of DELTA1. Essential mediator of somite segmentation and patterning.

Subcellular Location : Golgi apparatus membrane; Single-pass type II membrane protein

Post-translational modifications : A soluble form may be derived from the membrane form by proteolytic processing.

DISEASE : Defects in LFNG are the cause of spondylocostal dysostosis type 3 (SCDO3) [MIM:609813]. An autosomal recessive condition of variable severity associated with vertebral and rib segmentation defects. The main skeletal malformations include fusion of vertebrae, hemivertebrae, fusion of certain ribs, and other rib malformations. Deformity of the chest and spine (severe scoliosis, kyphoscoliosis and lordosis) is a natural consequence of the malformation and leads to a dwarf-like appearance. As the thorax is small, infants frequently have respiratory insufficiency and repeated respiratory infections resulting in life-threatening complications in the first year of life.

Similarity : Belongs to the glycosyltransferase 31 family.

Database links : UniProtKB/Swiss-Prot: Q8NES3.2

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