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货号： bs-11881R-PE-Cy7 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-11881R-PE-Cy7
- 英文名称
- Anti-Lunatic Fringe/PE-Cy7
- 中文名称
- PE-Cy7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
- 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: 3955Human
Entrez Gene: 16848Mouse
Entrez Gene: 170905Rat
Omim: 602576Human
SwissProt: Q8NES3Human
SwissProt: O09010Mouse
SwissProt: Q924T4Rat
Unigene: 159142Human
Unigene: 12834Mouse
Unigene: 127809Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.