Molecular weight (DA)
42kD
Immunogen
The antiserum was produced against synthesized peptide derived from human LFNG. AA range:121-170
Specificity
Lunatic Fringe Polyclonal Antibody detects endogenous levels of Lunatic Fringe protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications.
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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
Function
alternative products:Experimental confirmation may be lacking for some isoforms,catalytic activity:Transfers a beta-D-GlcNAc residue from UDP-D-GlcNAc to the fucose residue of a fucosylated protein acceptor.,caution:The sequence shown here is derived from an Ensembl automatic analysis pipeline and should be considered as preliminary data.,disease:Defects in LFNG are the cause of spondylocostal dysostosis autosomal recessive type 3 (SCDO3) [MIM:609813]. Autosomal recessive spondylocostal dysostosis is a rare 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
Protein name
Beta-1,3-N-acetylglucosaminyltransferase lunatic fringe
Abbreviation
Lunatic Fringe
Other name
LFNG; Beta-1; 3-N-acetylglucosaminyltransferase lunatic fringe; O-fucosylpeptide 3-beta-N-acetylglucosaminyltransferase
Fields
>>Other types of O-glycan biosynthesis;>>Notch signaling pathway;>>Human papillomavirus infection
Human protein sequence Database
Q8NES3
Mouse protein sequence database
O09010
Rat protein sequence database
Q924T4
Cellular localization
Golgi apparatus membrane ; Single-pass type II membrane protein .
Tissue expression
Kidney,
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