FITC标记的信号转导分子SCDO3抗体

FITC标记的信号转导分子SCDO3抗体

价格: ¥2980

品牌:LMAI Bio

货号:LM-11881R-FITC

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供应商 :上海联迈生物工程有限公司

库存 :大量

靶点 :详见说明书

级别 :1

目录编号 :LM-11881R-FITC

克隆性 :多克隆

抗原来源 :Rabbit

保质期 :1年

抗体英文名 :Anti-Lunatic Fringe/FITC

抗体名 :Anti-Lunatic Fringe/FITC

标记物 :FITC标记

宿主 :Human, Mouse, Rat, Chicken, Pig, Cow, Horse, Rabbit, Sheep,

适应物种 :Human, Mouse, Rat, Chicken, Pig, Cow, Horse, Rabbit, Sheep,

免疫原 :详见说明书

亚型 :IGg

形态 :粉末、液体、冻干粉

应用范围 :ICC=1:50-200 IF=1:50-200

浓度 :1mg/ml

保存条件 :-20 °C

规格 :100ul

FITC标记的信号转导分子SCDO3抗体
英文名称 Anti-Lunatic Fringe/FITC
中文名称 FITC标记的信号转导分子SCDO3抗体
别    名 LFNG; 3-N-acetylglucosaminyltransferase lunatic fringe; Beta-1; Beta-13-N-acetylglucosaminyltransferase lunatic fringe; lfng; LFNG_HUMAN; O-fucosylpeptide 3-beta-N-acetylglucosaminyltransferase; SCDO3.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  发育生物学  神经生物学  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Pig, Cow, Horse, Rabbit, Sheep, 
产品应用 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 42kDa
细胞定位 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SCDO3/Lunatic Fringe
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
产品介绍 background:
This gene is a member of the fringe gene family which also includes radical and manic fringe genes. They all encode evolutionarily conserved glycosyltransferases that act in the Notch signaling pathway to define boundaries during embryonic development. While their genomic structure is distinct from other glycosyltransferases, fringe proteins have a fucose-specific beta-1,3-N-acetylglucosaminyltransferase activity that leads to elongation of O-linked fucose residues on Notch, which alters Notch signaling. This gene product is predicted to be a single-pass type II Golgi membrane protein but it may also be secreted and proteolytically processed like the related proteins in mouse and Drosophila (PMID: 9187150). Mutations in this gene have been associated with autosomal recessive spondylocostal dysostosis 3. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009]

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:

Entrez Gene: 3955 Human

Entrez Gene: 16848 Mouse

Entrez Gene: 170905 Rat

Omim: 602576 Human

SwissProt: Q8NES3 Human

SwissProt: O09010 Mouse

SwissProt: Q924T4 Rat

Unigene: 159142 Human

Unigene: 12834 Mouse

Unigene: 127809 Rat



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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