Genomes and Genes
Gene Symbol: fibulin 1
Description: fibulin 1
Alias: FBLN, FIBL1, fibulin-1
Products: fibulin 1
- Greene L, Twal W, Duffy M, McDermott E, Hill A, O Higgins N, et al. Elevated expression and altered processing of fibulin-1 protein in human breast cancer. Br J Cancer. 2003;88:871-8 pubmed..43, P=0.008, n=36) expression in the tumour specimens. Taken together, these findings indicate that elevated expression and altered processing of fibulin-1 is associated with human breast cancer. ..
- Miosge N, Gotz W, Sasaki T, Chu M, Timpl R, Herken R. The extracellular matrix proteins fibulin-1 and fibulin-2 in the early human embryo. Histochem J. 1996;28:109-16 pubmed..The early embryonic expression of both fibulins indicates specific roles during organ development and, in particular, involvements in the differentiation of heart, skeletal and neuronal structures. ..
- Cheng Y, Jin H, Liu X, Siu J, Wong Y, Ng E, et al. Fibulin 1 is downregulated through promoter hypermethylation in gastric cancer. Br J Cancer. 2008;99:2083-7 pubmed publisher..In an effort to search for such genes aberrantly methylated in gastric cancer development, fibulin 1 (FBLN1) was found as a candidate TSG epigenetically downregulated in gastric cancer...
- Brown J, Wiedemann H, Timpl R. Protein binding and cell adhesion properties of two laminin isoforms (AmB1eB2e, AmB1sB2e) from human placenta. J Cell Sci. 1994;107 ( Pt 1):329-38 pubmed..No inhibition was observed with an Arg-Gly-Asp-containing peptide. ..
- Piscaglia F, Dudas J, Knittel T, Di Rocco P, Kobold D, Saile B, et al. Expression of ECM proteins fibulin-1 and -2 in acute and chronic liver disease and in cultured rat liver cells. Cell Tissue Res. 2009;337:449-62 pubmed publisher..Thus, fibulin-1 and -2 respond differentially to single and repeated damaging noxae, and their expression is differently present in liver cells. Expression of the fibulin-2 gene is regulated by TGF-beta1 in liver myofibroblasts. ..
- Du M, Fan X, Hong E, Chen J. Interaction of oncogenic papillomavirus E6 proteins with fibulin-1. Biochem Biophys Res Commun. 2002;296:962-9 pubmed..Significantly, overexpression of fibulin-1 specifically inhibited E6-mediated transformation. These results suggest that fibulin-1 plays an important role in the biological activities of E6. ..
- Kanda M, Nomoto S, Okamura Y, Hayashi M, Hishida M, Fujii T, et al. Promoter hypermethylation of fibulin 1 gene is associated with tumor progression in hepatocellular carcinoma. Mol Carcinog. 2011;50:571-9 pubmed publisher..Gene expression profiling showed the expression of fibulin 1 (FBLN1), located on 22q13, to be decreased in tumor tissue...
- Cangemi C, Skov V, Poulsen M, Funder J, Twal W, Gall M, et al. Fibulin-1 is a marker for arterial extracellular matrix alterations in type 2 diabetes. Clin Chem. 2011;57:1556-65 pubmed publisher..013). Fibulin-1 accumulates in the arterial wall and in plasma of patients with type 2 diabetes, and appears to be a factor associated with arterial extracellular matrix changes in type 2 diabetes. ..
- Kruger R, Schutte R, Huisman H, Argraves W, Rasmussen L, Olsen M, et al. NT-proBNP is associated with fibulin-1 in Africans: the SAfrEIC study. Atherosclerosis. 2012;222:216-21 pubmed publisher..012). Only Africans indicated a significant independent association between NT-proBNP and fibulin-1, suggesting that cardiovascular alterations are already present in this relatively young African population as opposed to Caucasians. ..
- Twal W, Czirok A, Hegedus B, Knaak C, Chintalapudi M, Okagawa H, et al. Fibulin-1 suppression of fibronectin-regulated cell adhesion and motility. J Cell Sci. 2001;114:4587-98 pubmed..This ability to influence signal transduction cascades that modulate the actin-myosin motor complex might be the basis for the effects of fibulin-1 on adhesion and motility. ..
- Argraves W, Tran H, Burgess W, Dickerson K. Fibulin is an extracellular matrix and plasma glycoprotein with repeated domain structure. J Cell Biol. 1990;111:3155-64 pubmed..The bulk of the remaining portion of the molecule is a series of nine EGF-like repeats. ..
- Pan T, Kostka G, Zhang R, Timpl R, Chu M. Complete exon-intron organization of the mouse fibulin-1 gene and its comparison with the human fibulin-1 gene. FEBS Lett. 1999;444:38-42 pubmed..By searching the database we identified most of the exons encoding the human fibulin-1 gene and showed that its exon-intron organization is similar to that of the mouse gene. ..
- Hayashido Y, Lucas A, Rougeot C, Godyna S, Argraves W, Rochefort H. Estradiol and fibulin-1 inhibit motility of human ovarian- and breast-cancer cells induced by fibronectin. Int J Cancer. 1998;75:654-8 pubmed..We conclude that both estradiol and fibulin-1 inhibit cancer cell motility in vitro and therefore have the potential to inhibit tumor invasion. ..
- Tran H, VanDusen W, Argraves W. The self-association and fibronectin-binding sites of fibulin-1 map to calcium-binding epidermal growth factor-like domains. J Biol Chem. 1997;272:22600-6 pubmed..However, unlike the self-association site in EGF-like modules 5 and 6, which was functional in the native protein, the amino-terminal site was cryptic and revealed only after the protein was denatured. ..
- Tran H, Tanaka A, Litvinovich S, Medved L, Haudenschild C, Argraves W. The interaction of fibulin-1 with fibrinogen. A potential role in hemostasis and thrombosis. J Biol Chem. 1995;270:19458-64 pubmed..The interaction between fibulin-1 and Fg highlights potential new roles for fibulin-1 in hemostasis as well as thrombosis. ..
- Tran H, Mattei M, Godyna S, Argraves W. Human fibulin-1D: molecular cloning, expression and similarity with S1-5 protein, a new member of the fibulin gene family. Matrix Biol. 1997;15:479-93 pubmed..In addition to this C-terminal similarity, S1-5 also contains repeated EGF-like modules and a conserved N-terminal element, thereby leading to the conclusion that S1-5 is a third member of the fibulin gene family. ..
- Clinton G, Rougeot C, Derancourt J, Roger P, Defrenne A, Godyna S, et al. Estrogens increase the expression of fibulin-1, an extracellular matrix protein secreted by human ovarian cancer cells. Proc Natl Acad Sci U S A. 1996;93:316-20 pubmed..The findings highlight a potential role for fibulin-1 in the spread of ovarian cancer in the peritoneal cavity and/or in distal metastases. ..
- Pan T, Kluge M, Zhang R, Mayer U, Timpl R, Chu M. Sequence of extracellular mouse protein BM-90/fibulin and its calcium-dependent binding to other basement-membrane ligands. Eur J Biochem. 1993;215:733-40 pubmed..The broad interaction repertoire of BM-90 is comparable to that of nidogen and both proteins may have similar roles as connecting elements in the extracellular matrix. ..
- Argraves W, Dickerson K, Burgess W, Ruoslahti E. Fibulin, a novel protein that interacts with the fibronectin receptor beta subunit cytoplasmic domain. Cell. 1989;58:623-9 pubmed..Therefore this protein, which we term fibulin, interacts with the fibronectin receptor in vitro and associates with the receptor in vivo. Fibulin is a potential mediator of interactions between adhesion receptors and the cytoskeleton. ..
- Roark E, Keene D, Haudenschild C, Godyna S, Little C, Argraves W. The association of human fibulin-1 with elastic fibers: an immunohistological, ultrastructural, and RNA study. J Histochem Cytochem. 1995;43:401-11 pubmed..g., that it is a structural protein that contributes to the elastic properties of connective tissue fibers or that is involved with the process of fibrogenesis. ..
- Godyna S, Diaz Ricart M, Argraves W. Fibulin-1 mediates platelet adhesion via a bridge of fibrinogen. Blood. 1996;88:2569-77 pubmed..This bridging phenomenon may represent a general mechanism by which platelets interact with exposed subendothelial matrices following vascular injury. ..
- Garcia A, Eiras S, Parguiña A, Alonso J, Rosa I, Salgado Somoza A, et al. High-resolution two-dimensional gel electrophoresis analysis of atrial tissue proteome reveals down-regulation of fibulin-1 in atrial fibrillation. Int J Cardiol. 2011;150:283-90 pubmed publisher..The lower expression of the structural protein fibulin-1 in atrial tissue from AF patients might reflect the myocardial structural changes that take place in the arrhythmia. ..
- Lee N, Rodriguez Manzaneque J, Thai S, Twal W, Luque A, Lyons K, et al. Fibulin-1 acts as a cofactor for the matrix metalloprotease ADAMTS-1. J Biol Chem. 2005;280:34796-804 pubmed..Together, these findings indicate that fibulin-1 is a new regulator of ADAMTS-1-mediated proteoglycan proteolysis and thus may play an important role in proteoglycan turnover in tissues where there is overlapping expression. ..
- Sasaki T, Göhring W, Miosge N, Abrams W, Rosenbloom J, Timpl R. Tropoelastin binding to fibulins, nidogen-2 and other extracellular matrix proteins. FEBS Lett. 1999;460:280-4 pubmed..The colocalization of these proteins emphasizes the potential for in vivo interactions. ..
- Hang Pham L, Yoo Y, Park S, Back S, Kim S, BjÃ¸rge I, et al. Investigating the effect of fibulin-1 on the differentiation of human nasal inferior turbinate-derived mesenchymal stem cells into osteoblasts. J Biomed Mater Res A. 2017;105:2291-2298 pubmed publisher..2017 Wiley Periodicals, Inc. J Biomed Mater Res Part A: 105A: 2291-2298, 2017. ..
- Xiao W, Wang J, Li H, Guan W, Xia D, Yu G, et al. Fibulin-1 is down-regulated through promoter hypermethylation and suppresses renal cell carcinoma progression. J Urol. 2013;190:291-301 pubmed publisher..Fibulin-1 is down-regulated in renal cell carcinoma through promoter hypermethylation. It functions as a tumor suppressor and angiogenesis inhibitor in renal cell carcinoma. ..
- Guo J, Cheng C, Chen C, Xing X, Xu G, Feng J, et al. Overexpression of Fibulin-5 Attenuates Ischemia/Reperfusion Injury After Middle Cerebral Artery Occlusion in Rats. Mol Neurobiol. 2016;53:3154-3167 pubmed publisher..confirmed brain edema reduction and decreased BBB disruption in fibulin-5 overexpression recombinant adenovirus (Ad-FBLN) treated rats...
- Paapstel K, Zilmer M, Eha J, Tootsi K, Piir A, Kals J. Association Between Fibulin-1 and Aortic Augmentation Index in Male Patients with Peripheral Arterial Disease. Eur J Vasc Endovasc Surg. 2016;51:76-82 pubmed publisherFibulin-1 (FBLN-1), a newly identified biomarker for vascular stiffness in type 2 diabetes, may participate in the pathophysiological processes leading to progression of arterial stiffness in atherosclerosis...
- Rapisarda V, Ledda C, Migliore M, Salemi R, Musumeci A, Bracci M, et al. FBLN-3 as a biomarker of pleural plaques in workers occupationally exposed to carcinogenic fibers: a pilot study. Future Oncol. 2015;11:35-7 pubmed publisherb>FBLN-3 has recently been proposed as a biomarker for malignant mesothelioma. A significantly increased standardized mortality rate from malignant mesothelioma has been reported in Biancavilla, Italy...
- Motalebzadeh J, Mahjoubi F, Nafissi N, Hashemian M, Taheri M, Hosseinpour Y. FBLN-4 and BCRP genes as two prognostic markers are downregulated in breast cancer tissue. Cancer Biomark. 2017;19:51-55 pubmed publisherFibulin-4 (FBLN-4) is an extracellular glycoprotein that is upregulated in some cancer and is khown as prognostic marker in ovarian and cervical cancer...
- Weigell Weber M, Sarra G, Kotzot D, Sandkuijl L, Messmer E, Hergersberg M. Genomewide homozygosity mapping and molecular analysis of a candidate gene located on 22q13 (fibulin-1) in a previously undescribed vitreoretinal dystrophy. Arch Ophthalmol. 2003;121:1184-8 pubmed..A genomewide homozygosity mapping analysis supported the hypothesis that the gene responsible for a unique vitreoretinal dystrophy is located on chromosome 22q13. No obviously pathogenic mutation was found in the candidate gene, FBLN1. ..
- Fujimoto N, Terlizzi J, Brittingham R, Fertala A, McGrath J, Uitto J. Extracellular matrix protein 1 interacts with the domain III of fibulin-1C and 1D variants through its central tandem repeat 2. Biochem Biophys Res Commun. 2005;333:1327-33 pubmed
- Castoldi M, Chu M. Structural and functional characterization of the human and mouse fibulin-1 gene promoters: role of Sp1 and Sp3. Biochem J. 2002;362:41-50 pubmed..The results demonstrate the functional importance of Sp1 and Sp3 in regulating the expression of the fibulin-1 gene. ..
- Lv F, Peng Y, Lim F, Sun Y, Lv M, Zhou L, et al. Matrix metalloproteinase 12 is an indicator of intervertebral disc degeneration co-expressed with fibrotic markers. Osteoarthritis Cartilage. 2016;24:1826-1836 pubmed publisher..Overall, our findings implicate an impact of MMP12 in disc cell homeostasis. The precise role of MMP12 in IVD degeneration warrants further investigation. ..
- Tang J, Liu J, Liu X, Liang X, Cai X. Effect of fibulin-5 on adhesion, migration and invasion of hepatocellular carcinoma cells via an integrin-dependent mechanism. World J Gastroenterol. 2015;21:11127-40 pubmed publisherTo elucidate the role of fibulin-5 (FBLN-5) as a suppressor of hepatocellular carcinoma (HCC) cell metastasis via integrin...
- Kruger R, Rasmussen L, Argraves W, Eugen Olsen J, Nielsen O, Blyme A, et al. Extracellular matrix biomarker, fibulin-1, is closely related to NT-proBNP and soluble urokinase plasminogen activator receptor in patients with aortic valve stenosis (the SEAS study). PLoS ONE. 2014;9:e101522 pubmed publisher
- Shen L, Liao L, Chen C, Guo Y, Song D, Wang Y, et al. Proteomics Analysis of Blood Serums from Alzheimer's Disease Patients Using iTRAQ Labeling Technology. J Alzheimers Dis. 2017;56:361-378 pubmed publisher..Specifically, our results strengthened the crucial role of A? metabolism and blood coagulation in AD pathogenesis and proteins related to these two processes may be used as peripheral blood biomarkers for AD. ..
- Mokwatsi G, Schutte A, Kruger R. A biomarker of tissue damage, lactate dehydrogenase, is associated with fibulin-1 and oxidative stress in blacks: the SAfrEIC study. Biomarkers. 2016;21:48-55 pubmed publisher..11; p = 0.30) in blacks only. Our finding suggests that increased circulating levels of LDH may be due to early ECM remodeling and oxidative stress in blacks that are subjected to detrimental and uncontrolled lifestyle risk factors. ..
- Holmila R, Sklias A, Muller D, Degli Esposti D, Guilloreau P, McKay J, et al. Targeted deep sequencing of plasma circulating cell-free DNA reveals Vimentin and Fibulin 1 as potential epigenetic biomarkers for hepatocellular carcinoma. PLoS ONE. 2017;12:e0174265 pubmed publisher..Also, the potential to investigate methylation patterns in cfDNA could bring new strategies for HCC detection and monitoring high-risk groups and response to treatment. ..
- Wu C, Liu H, Zhang F, Shao W, Yang L, Ning Y, et al. Long noncoding RNA expression profile reveals lncRNAs signature associated with extracellular matrix degradation in kashin-beck disease. Sci Rep. 2017;7:17553 pubmed publisher..In the network, FBLN1 and LAMA 4 were the core genes with the highest significance. These novel findings indicate that lncRNAs may play a role in extracellular matrix destruction in KBD. ..
- Oxlund C, Cangemi C, Henriksen J, Jacobsen I, Gram J, Schousboe K, et al. Low-dose spironolactone reduces plasma fibulin-1 levels in patients with type 2 diabetes and resistant hypertension. J Hum Hypertens. 2015;29:28-32 pubmed publisher..This supports the hypothesis that the antihypertensive effect of the mineralocorticoid receptor blocker in part may be due to regression of vascular remodeling. ..
- Bohlega S, Al Ajlan H, Al Saif A. Mutation of fibulin-1 causes a novel syndrome involving the central nervous system and connective tissues. Eur J Hum Genet. 2014;22:640-3 pubmed publisher..Our findings emphasize the crucial role fibulin-1 has in development of the central nervous system and various connective tissues. ..
- Williams S, Schwarzbauer J. A shared mechanism of adhesion modulation for tenascin-C and fibulin-1. Mol Biol Cell. 2009;20:1141-9 pubmed publisher..Therefore, blockade of HepII-mediated signaling by competitive binding of fibulin-1 or tenascin-C represents a shared mechanism of adhesion modulation among disparate modulatory proteins. ..
- Mattei M, Pan T, Zhang R, Timpl R, Chu M. The fibulin-1 gene (FBLN1) is located on human chromosome 22 and on mouse chromosome 15. Genomics. 1994;22:437-8 pubmed..The gene was assigned to the q13.2-q13.3 region of human chromosome 22 and to the E-F band of mouse chromosome 15. This finding extends the evolutionary conservation between human chromosome 22 and mouse chromosome 15. ..
- DiScipio R, Liddington R, Schraufstatter I. Fibulin-1 purification from human plasma using affinity chromatography on Factor H-Sepharose. Protein Expr Purif. 2016;121:118-24 pubmed publisher..The procedure described is the first to our knowledge that enables a large scale purification of Fibulin-1 from human plasma. ..
- Wen Y, Giardina S, Hamming D, Greenman J, Zachariah E, Bacolod M, et al. GROalpha is highly expressed in adenocarcinoma of the colon and down-regulates fibulin-1. Clin Cancer Res. 2006;12:5951-9 pubmed..Targeting GRO proteins may provide new opportunities for treatment of colon cancer. ..
- Palumbo P, Accadia M, Leone M, Palladino T, Stallone R, Carella M, et al. Clinical and molecular characterization of an emerging chromosome 22q13.31 microdeletion syndrome. Am J Med Genet A. 2017;: pubmed publisher..31 microdeletions facilitating genotype-phenotype correlations. Additional cases are needed to corroborate our hypothesis and confirm genotype-phenotype correlations of this emerging syndrome...
- Ohsawa I, Takamura C, Kohsaka S. Fibulin-1 binds the amino-terminal head of beta-amyloid precursor protein and modulates its physiological function. J Neurochem. 2001;76:1411-20 pubmed..Furthermore, fibulin-1 blocks sAPP-mediated proliferation of primary cultured rat neural stem cells. These results suggest that fibulin-1 may play a significant role in modulating the neurotrophic activities of APP. ..
- Nakamoto T, Okada H, Nakajima T, Ikuta A, Yasuda K, Kanzaki H. Progesterone induces the fibulin-1 expression in human endometrial stromal cells. Hum Reprod. 2005;20:1447-55 pubmed..By contrast, beta-estradiol alone did not increase the fibulin-1 mRNA expression. These results suggest that fiblin-1 is an important molecule that mediates progesterone action in human ESC differentiation towards implantation. ..
- Korenberg J, Chen X, Tran H, Argraves W. Localization of the human gene for fibulin-1 (FBLN1) to chromosome band 22q13.3. Cytogenet Cell Genet. 1995;68:192-3 pubmed..3). The assignment of the chromosomal map position for the fibulin-1 gene will aid in the evaluation of its potential roles in human connective tissue and blood diseases. ..
- Tian M, Wang J, Wei Y, Lu Q, Huang B. Serum and vitreous fibulin-1 concentrations in patients with diabetic retinopathy. J Investig Med. 2016;64:1209-12 pubmed publisher..Serum and vitreous fibulin-1 concentrations are elevated under DR condition. ..
- Wooten E, Iyer L, Montefusco M, Hedgepeth A, Payne D, Kapur N, et al. Application of gene network analysis techniques identifies AXIN1/PDIA2 and endoglin haplotypes associated with bicuspid aortic valve. PLoS ONE. 2010;5:e8830 pubmed publisher..Further, these studies demonstrate that leveraging existing expression and genomic data in the context of GWAS studies can identify biologically relevant genes and pathways associated with a congenital heart defect. ..
- Laugesen E, Høyem P, Christiansen J, Knudsen S, Hansen K, Argraves W, et al. Plasma levels of the arterial wall protein fibulin-1 are associated with carotid-femoral pulse wave velocity: a cross-sectional study. Cardiovasc Diabetol. 2013;12:107 pubmed publisher..Further studies are needed to determine the exact role of fibulin-1 in arterial stiffness and cardiovascular risk in patients with type 2 diabetes. ..
- Lau J, Oliver B, Baraket M, Beckett E, Hansbro N, Moir L, et al. Fibulin-1 is increased in asthma--a novel mediator of airway remodeling?. PLoS ONE. 2010;5:e13360 pubmed publisher..In a mouse model of AHR, treatment with an AO inhibited the development of AHR to methacholine. Our data collectively suggest fibulin-1C may be worthy of further investigation as a target for airway remodeling in asthma. ..
- Penner A, Rock M, Kielty C, Shipley J. Microfibril-associated glycoprotein-2 interacts with fibrillin-1 and fibrillin-2 suggesting a role for MAGP-2 in elastic fiber assembly. J Biol Chem. 2002;277:35044-9 pubmed..The results also demonstrate the utility of the yeast two-hybrid system to study protein-protein interactions of the extracellular matrix. ..
- Brooke J, Cha J, Eidels L. Latent transforming growth factor beta-binding protein-3 and fibulin-1C interact with the extracellular domain of the heparin-binding EGF-like growth factor precursor. BMC Cell Biol. 2002;3:2 pubmed..The interactions of the extracellular domain of proHB-EGF with LTBP-3 and fibulin-1C suggest novel functions for HB-EGF between cell and tissue surfaces. ..
- Greenwood T, Akiskal H, Akiskal K, Kelsoe J. Genome-wide association study of temperament in bipolar disorder reveals significant associations with three novel Loci. Biol Psychiatry. 2012;72:303-10 pubmed publisher..These results suggest that aspects of temperament might define subtypes of BD that are more clinically and genetically homogenous, which might aid in the identification of predisposing genetic variants. ..
- Overgaard M, Cangemi C, Jensen M, Argraves W, Rasmussen L. Total and isoform-specific quantitative assessment of circulating fibulin-1 using selected reaction monitoring MS and time-resolved immunofluorometry. Proteomics Clin Appl. 2015;9:767-75 pubmed publisher..While the correlation between these methods was acceptable, low correlation was obtained between the commercial CircuLex assay and SRM-MS or TRIFMA. ..
- Xiao W, Wang J, Li H, Xia D, Yu G, Yao W, et al. Fibulin-1 is epigenetically down-regulated and related with bladder cancer recurrence. BMC Cancer. 2014;14:677 pubmed publisher..Altogether, our results indicated that fibulin-1 expression is associated with NMIBC grade and recurrence, it is epigenetically down-regulated and functions as a tumor suppressor gene and angiogenesis inhibitor in bladder cancer. ..
- Scholze A, Bladbjerg E, Sidelmann J, Diederichsen A, Mickley H, Nybo M, et al. Plasma concentrations of extracellular matrix protein fibulin-1 are related to cardiovascular risk markers in chronic kidney disease and diabetes. Cardiovasc Diabetol. 2013;12:6 pubmed publisher..Furthermore, fibulin-1 levels were associated with hemodynamic cardiovascular risk markers. Fibulin-1 is a candidate in the pathogenesis of cardiovascular disease observed in chronic kidney disease and diabetes. ..
- Perbal B, Martinerie C, Sainson R, Werner M, He B, Roizman B. The C-terminal domain of the regulatory protein NOVH is sufficient to promote interaction with fibulin 1C: a clue for a role of NOVH in cell-adhesion signaling. Proc Natl Acad Sci U S A. 1999;96:869-74 pubmed..These results set the stage for a study of NOVH-fibulin 1C interactions and their potential significance in cell-adhesion signaling in normal and pathological conditions. ..