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fibroblast growth factor 9; FGF-9; Glia-activating factor; GAF; heparin-binding growth factor 9; HBGF-9 (FGF9)
Function:
- role in regulation of embryonic development, cell proliferation, cell differentiation & cell migration
- may have a role in glial cell growth & differentiation during development, gliosis during repair & regeneration of brain tissue after damage, differentiation & survival of neuronal cells, & growth stimulation of glial tumors
- three molecular species were found (30 kD, 29 kD & 25 kDa), cleaved at Leu-4, Val-13 & Ser-34 respectively
- the smaller ones might be products of proteolytic digestion
- furthermore, there may be a functional signal sequence in the 30 kDa species which is uncleavable in the secretion step
- interacts with FGFR1, FGFR2, FGFR3 & FGFR4
- affinity between fibroblast growth factors (FGFs) & their receptors is increased by heparan sulfate glycosaminoglycans that function as coreceptors
Structure:
- N-glycosylated
- monomer, homodimer
- belongs to the heparin-binding growth factors family
Compartment: secreted
Expression: Glial cells
Pathology:
- defects in FGF9 are the cause of multiple synostoses syndrome type 3
- the Asn-99 mutation is associated with impaired FGF signaling, as evidenced by diminished activity of the MAPK1/MAPK2 pathway & decreases CTNNB1 & MYC expression when compared with wild-type protein
- binding of mutant protein to the receptor FGFR3 is severely impaired, although homodimerization of mutant to itself or wild-type is not detectably affected
General
fibroblast growth factor
glycoprotein
Properties
SIZE: entity length = 208 aa
MW = 23 kD
COMPARTMENT: extracellular compartment
MOTIF: N-glycosylation site {N79}
SECRETED-BY: endothelial cell
Database Correlations
OMIM correlations
MORBIDMAP 600921
UniProt P31371
Pfam PF00167
Entrez Gene 2254
Kegg hsa:2254
References
- UniProt :accession P31371
- R&D systems' cytokine source book: FGF-9
http://www.rndsystems.com/molecule_detail.aspx?m=1436
- NIEHS-SNPs
http://egp.gs.washington.edu/data/fgf9/