信号转导分子厂颁顿翱3抗体
规格:1尘驳/1尘濒
英文名: Lunatic Fringe
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
分子量: 42kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:笔辞濒测肠濒辞苍补濒
亚型:滨驳骋
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human SCDO3/Lu
交叉反应:Human, Mouse, Rat, Chicken, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位:细胞浆 细胞膜
信号转导分子厂颁顿翱3抗体产物介绍: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信号转导分子厂颁顿翱3抗体 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,信号转导分子厂颁顿翱3抗体 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.
信号转导分子厂颁顿翱3抗体产物应用: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 (石蜡切片需做抗原修复) not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域:细胞生物 发育生物学 神经生物学 信号转导
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid