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lunatic fringe; beta-1,3-N-acetylglucosaminyltransferase lunatic fringe; O-fucosylpeptide 3-beta-N-acetylglucosaminyltransferase (LFNG)
Function:
- glycosyltransferase that initiates the elongation of O-linked fucose residues attached to EGF-like repeats in the extracellular domain of notch molecules.
- Lunatic fringe modifies Notch2 between the N-terminus & the 15th EGF repeat differing from manic fringe which modifies Notch2 between the 23rd & 29th EGF repeats thus resulting in a different modulation of Notch2 signalling
- decreases binding of JAGGED1 to NOTCH2
- does not effect binding of DELTA1
- mediator of somite segmentation & patterning (putative)
- transfers a beta-D-GlcNAc residue from UDP-D-GlcNAc to the fucose residue of a fucosylated protein acceptor
- soluble form may be derived from the membrane form by proteolytic processing (putative)
Cofactor: Mn+2 (putative)
Structure: belongs to the glycosyltransferase 31 family
Compartment:
- Golgi membrane
- single-pass type 2 membrane protein (putative)
Alternative splicing: named isoforms=3
Expression:
- oscillations of lunatic fringe transcripts occur during somitogenesis controlled at the transcriptional level by clock elements in the promoter region of the gene
- Notch signalling activates lunatic fringe transcription but is not an inherent component of the oscillator
Pathology:
- defects in LFNG are the cause of spondylocostal dysostosis type 3
General
fringe homolog protein
secreted protein
Properties
SIZE: entity length = 379 aa
MW = 42 kD
COMPARTMENT: golgi
MOTIF: transmembrane domain {9-29}
proteolytic site {86-87}
binding site
SITE: 129-129
FOR-BINDING-OF: Substrate
N-glycosylation site {N167}
cysteine residue {C168}
MODIFICATION: cysteine residue {C179}
cysteine residue {C179}
MODIFICATION: cysteine residue {C168}
cysteine residue {C197}
MODIFICATION: cysteine residue {C260}
binding site
SITE: 201-201
FOR-BINDING-OF: Substrate
manganese [Mn]-binding site
SITE: 202-202
cysteine residue {C260}
MODIFICATION: cysteine residue {C197}
aspartate residue {D290}
manganese [Mn]-binding site
SITE: 314-314
cysteine residue {C364}
MODIFICATION: cysteine residue {C373}
cysteine residue {C373}
MODIFICATION: cysteine residue {C364}
Database Correlations
OMIM correlations
MORBIDMAP 602576
UniProt Q8NES3
Pfam PF02434
Entrez Gene 3955
Kegg hsa:3955
ENZYME 2.4.1.222
References
- Pourquie O.
Vertebrate segmentation: lunatic transcriptional regulation.
Current Biology 12:R699-R701, 2002
PMID: 12401188
- Shimizu K et al.
Manic fringe and lunatic fringe modify different sites of the
Notch2 extracellular region, resulting in different signaling
modulation.
J Biol Chem 276: 25753-8, 2001.
PMID: 11346656
- Entrez Gene :accession 3955