LFNG / Lunatic Fringe, Polyclonal Antibody

Anti-LFNG / Lunatic Fringe Antibody (aa93-122) IHC-plus; Lunatic Fringe; Human LFNG; Lunatic Fringe

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Human LFNG

Immunoaffinity Purified

PBS, 0.09% sodium azide

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Small volumes of anti-LFNG antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Immunohistochemistry (IHC - Paraffin), Western Blot (WB), Flow Cytometry (FC/FACS)

Flo (1:10 - 1:50), IHC-P (1:100), WB (1:1000)

Anti-LFNG / Lunatic Fringe antibody IHC staining of human liver. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval.

Anti-LFNG / Lunatic Fringe antibody IHC staining of human kidney. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody dilution 1:100.

LFNG Antibody (Center) flow cytometry of HL-60 cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.

LFNG Antibody (Center) Western blot of HL-60,K562,HeLa cell line lysates (35 ug/lane). This demonstrates the LFNG antibody detected the LFNG protein (arrow).

35,266 Da

LFNG O-fucosylpeptide 3-beta-N-acetylglucosaminyltransferase

beta-1,3-N-acetylglucosaminyltransferase lunatic fringe

Beta-1,3-N-acetylglucosaminyltransferase lunatic fringe

LFNG_HUMAN

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]

LFNG: 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. Defects in LFNG are the cause of spondylocostal dysostosis type 3 (SCDO3). 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. Belongs to the glycosyltransferase 31 family. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 2.4.1.222; Transferase; Membrane protein, integral; Cell cycle regulation

Chromosomal Location of Human Ortholog: 7p22.2

Cellular Component: extracellular region; integral to Golgi membrane; vesicle

Molecular Function: metal ion binding; O-fucosylpeptide 3-beta-N-acetylglucosaminyltransferase activity

Biological Process: compartment specification; organ morphogenesis; somitogenesis; Notch signaling pathway; regulation of Notch signaling pathway; ovarian follicle development; positive regulation of protein binding; metabolic process; female meiosis; regulation of somitogenesis; positive regulation of Notch signaling pathway

Disease: Spondylocostal Dysostosis 3, Autosomal Recessive

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