FBLN2 explained

Fibulin-2 is a protein that in humans is encoded by the FBLN2 gene.^[1]

This gene encodes an extracellular matrix protein, which belongs to the fibulin family. This protein binds various extracellular ligands and calcium. It may play a role during organ development, in particular, during the differentiation of heart, skeletal and neuronal structures. Alternatively spliced transcript variants encoding different isoforms have been identified.^[2]

Its role as a biomarker for meningiomas (a common tumour affecting the central nervous system) was recently described where a blood test can predict whether patients have a grade II meningiomas (poor outcome) and not a grade I meningioma (better outcome), without the need for a surgical biopsy.^[3]

Interactions

FBLN2 has been shown to interact with Laminin, alpha 1,^{[4] [5]} Laminin, alpha 5^[4] and Perlecan.^{[6] [7]}

Further reading

• Sasaki T, Göhring W, Pan TC . Binding of mouse and human fibulin-2 to extracellular matrix ligands. . J. Mol. Biol. . 254 . 5 . 892–9 . 1996 . 7500359 . 10.1006/jmbi.1995.0664 . etal.
• Pan TC, Sasaki T, Zhang RZ . Structure and expression of fibulin-2, a novel extracellular matrix protein with multiple EGF-like repeats and consensus motifs for calcium binding . J. Cell Biol. . 123 . 5 . 1269–77 . 1994 . 8245130 . 10.1083/jcb.123.5.1269 . 2119879 . etal.
• Reinhardt DP, Sasaki T, Dzamba BJ . Fibrillin-1 and fibulin-2 interact and are colocalized in some tissues . J. Biol. Chem. . 271 . 32 . 19489–96 . 1996 . 8702639 . 10.1074/jbc.271.32.19489 . etal. free .
• Miosge N, Götz W, Sasaki T . The extracellular matrix proteins fibulin-1 and fibulin-2 in the early human embryo . Histochem. J. . 28 . 2 . 109–16 . 1996 . 8737292 . 10.1007/BF02331415 . 25996538 . etal.
• Collod G, Chu ML, Sasaki T . Fibulin-2: genetic mapping and exclusion as a candidate gene in Marfan syndrome type 2 . Eur. J. Hum. Genet. . 4 . 5 . 292–5 . 1997 . 8946175 . 10.1159/000472216. 9891855 . etal.
• Utani A, Nomizu M, Yamada Y . Fibulin-2 binds to the short arms of laminin-5 and laminin-1 via conserved amino acid sequences . J. Biol. Chem. . 272 . 5 . 2814–20 . 1997 . 9006922 . 10.1074/jbc.272.5.2814 . free .
• Sasaki T, Mann K, Wiedemann H . Dimer model for the microfibrillar protein fibulin-2 and identification of the connecting disulfide bridge . EMBO J. . 16 . 11 . 3035–43 . 1997 . 9214621 . 10.1093/emboj/16.11.3035 . 1169922 . etal.
• Brown JC, Sasaki T, Göhring W . The C-terminal domain V of perlecan promotes beta1 integrin-mediated cell adhesion, binds heparin, nidogen and fibulin-2 and can be modified by glycosaminoglycans . Eur. J. Biochem. . 250 . 1 . 39–46 . 1998 . 9431988 . 10.1111/j.1432-1033.1997.t01-1-00039.x . etal. free .
• Raghunath M, Tschödrich-Rotter M, Sasaki T . Confocal laser scanning analysis of the association of fibulin-2 with fibrillin-1 and fibronectin define different stages of skin regeneration . J. Invest. Dermatol. . 112 . 1 . 97–101 . 1999 . 9886271 . 10.1046/j.1523-1747.1999.00483.x . etal. free .
• Kuivaniemi H, Marshall A, Ganguly A . Fibulin-2 exhibits high degree of variability, but no structural changes concordant with abdominal aortic aneurysms . Eur. J. Hum. Genet. . 6 . 6 . 642–6 . 1999 . 9887386 . 10.1038/sj.ejhg.5200245 . etal. free .
• Sasaki T, Göhring W, Miosge N . Tropoelastin binding to fibulins, nidogen-2 and other extracellular matrix proteins . FEBS Lett. . 460 . 2 . 280–4 . 1999 . 10544250 . 10.1016/S0014-5793(99)01362-9 . 25139630 . etal. free .
• Gu YC, Nilsson K, Eng H, Ekblom M . Association of extracellular matrix proteins fibulin-1 and fibulin-2 with fibronectin in bone marrow stroma . Br. J. Haematol. . 109 . 2 . 305–13 . 2000 . 10848816 . 10.1046/j.1365-2141.2000.02011.x . 37405859 . free .
• Talts JF, Sasaki T, Miosge N . Structural and functional analysis of the recombinant G domain of the laminin alpha4 chain and its proteolytic processing in tissues . J. Biol. Chem. . 275 . 45 . 35192–9 . 2001 . 10934193 . 10.1074/jbc.M003261200 . etal. free .
• Olin AI, Mörgelin M, Sasaki T . The proteoglycans aggrecan and Versican form networks with fibulin-2 through their lectin domain binding . J. Biol. Chem. . 276 . 2 . 1253–61 . 2001 . 11038354 . 10.1074/jbc.M006783200 . etal. free .
• Hopf M, Göhring W, Mann K, Timpl R . Mapping of binding sites for nidogens, fibulin-2, fibronectin and heparin to different IG modules of perlecan . J. Mol. Biol. . 311 . 3 . 529–41 . 2001 . 11493006 . 10.1006/jmbi.2001.4878 .
• Hunzelmann N, Nischt R, Brenneisen P . Increased deposition of fibulin-2 in solar elastosis and its colocalization with elastic fibres . Br. J. Dermatol. . 145 . 2 . 217–22 . 2001 . 11531782 . 10.1046/j.1365-2133.2001.04337.x . 45863539 . etal.
• Sasaki T, Göhring W, Mann K . Short arm region of laminin-5 gamma2 chain: structure, mechanism of processing and binding to heparin and proteins . J. Mol. Biol. . 314 . 4 . 751–63 . 2002 . 11733994 . 10.1006/jmbi.2001.5176 . etal.
• Bengtsson E, Mörgelin M, Sasaki T . The leucine-rich repeat protein PRELP binds perlecan and collagens and may function as a basement membrane anchor . J. Biol. Chem. . 277 . 17 . 15061–8 . 2002 . 11847210 . 10.1074/jbc.M108285200 . etal. free .
• Strausberg RL, Feingold EA, Grouse LH . Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences . Proc. Natl. Acad. Sci. U.S.A. . 99 . 26 . 16899–903 . 2003 . 12477932 . 10.1073/pnas.242603899 . 139241 . etal. 2002PNAS...9916899M . free .

Notes and References

1. Zhang RZ, Pan TC, Zhang ZY, Mattei MG, Timpl R, Chu ML . Fibulin-2 (FBLN2): human cDNA sequence, mRNA expression, and mapping of the gene on human and mouse chromosomes . Genomics . 22 . 2 . 425–30 . January 1995 . 7806230 . 10.1006/geno.1994.1404 .
2. "FBLN2 fibulin 2 [Homo sapiens (human) ]"
3. Sofela . Agbolahan A. . Hilton . David A. . Ammoun . Sylwia . Baiz . Daniele . Adams . Claire L. . Ercolano . Emanuela . Jenkinson . Michael D. . Kurian . Kathreena M. . Teo . Mario . Whitfield . Peter C. . Sahm . Felix . Hanemann . C. Oliver . Fibulin-2: A Novel Biomarker for Differentiating Grade II from Grade I Meningiomas . International Journal of Molecular Sciences . 8 January 2021 . 22 . 2 . 560 . 10.3390/ijms22020560 . 33429944 . 7827565 . free .
4. Utani . A . Nomizu M . Yamada Y . January 1997 . Fibulin-2 binds to the short arms of laminin-5 and laminin-1 via conserved amino acid sequences . J. Biol. Chem. . 272 . 5 . 2814–20 . 9006922 . 10.1074/jbc.272.5.2814 . free .
5. Talts . J F . Sasaki T . Miosge N . Göhring W . Mann K . Mayne R . Timpl R . November 2000 . Structural and functional analysis of the recombinant G domain of the laminin alpha4 chain and its proteolytic processing in tissues . J. Biol. Chem. . 275 . 45 . 35192–9 . 10934193 . 10.1074/jbc.M003261200 . free .
6. Hopf . M . Göhring W . Mann K . Timpl R . August 2001 . Mapping of binding sites for nidogens, fibulin-2, fibronectin and heparin to different IG modules of perlecan . J. Mol. Biol. . 311 . 3 . 529–41 . 11493006 . 10.1006/jmbi.2001.4878 .
7. Sasaki . T . Göhring W . Pan T C . Chu M L . Timpl R . December 1995 . Binding of mouse and human fibulin-2 to extracellular matrix ligands . J. Mol. Biol. . 254 . 5 . 892–9 . 7500359 . 10.1006/jmbi.1995.0664 .