Property Summary

NCBI Gene PubMed Count 88
PubMed Score 204.17
PubTator Score 113.83

Knowledge Summary

Patent

No data available

TINX Plot

  Disease (7)

Disease Target Count Z-score Confidence
Cutis laxa 28 6.603 3.3

Expression

  Differential Expression (22)

Protein-protein Interaction (6)

Gene RIF (64)

PMID Text
26577699 Data indicate that Fbln5 promotes PDAC progression by functioning as a molecular rheostat that modulates cell-ECM interactions to reduce ROS production, and thus tip the balance in favor of tumor cell survival and treatment-refractory disease.
26506560 FBLN5 mRNA expression is upregulated in response to cAMP-mediated decidualization of primary human endometrial stromal cells, although FBLN5 itself does not enhance decidualization
26494967 Lower FBLN-5 expression is an important indicator of poor survival in hepatocellular carcinoma. FBLN-5 inhibits HCC adhesion/motility via integrin-dependent mechanism.
26251522 Fibulin-5 is significantly down-regulated in ovarian carcinoma and acts as a tumour suppressor by inhibiting the migration and invasion of ovarian cancer cells.
26095157 study demonstrates the pivotal role of the extracellular protein, fibulin-5, on the adhesion and proliferation of human keloid-derived cells, through binding to integrin beta-1
25909283 Data suggest that fibulin-5 functions as a metastasis suppressor in lung cancer by modulating tumor microenvironment to suppress Wnt/beta-catenin signaling.
25891043 Fibulin-5 may be implicated in the aetiology of rectal prolapse in a subgroup of young male patients.
25845228 Fibulin-5 plays critical roles in proliferation, migration and invasion of certain tumors, and the effect of fibulin-5 on tumorigenesis appears to be largely context-dependent. (Review)
25807371 Fibulin-5 expression is a disease marker of hepatic fibrosis.
25792650 Up-regulation of elastin and fibulin-5 mRNA levels in ICA were strongly correlated with family history of cardiovascular disease when compared to CCA
25684523 Loss of FBLN5 expression is associated with glioma.
25494879 These results indicate that Fibulin-5 inhibits hepatocellular carcinoma invasion and metastasis by suppressing matrix metalloproteinase-7 expression.
24990664 We identified a clear gene signature for elastogenesis in COPD and propose FBLN5 as a novel player in tissue repair in COPD.
24917111 Current data provide...strong evidence that common single-nucleotide polymorphisms of the FBLN5 gene are associated with pelvic organ prolapse especially after pelvic floor injury.
24386352 fibulin-5 overexpression in NPC specimens correlated with advanced tumor metastasis indicating a poor 5-year overall survival
24148803 Overall the results indicate that LTBP-2 may have a negative regulatory role during elastic fiber assembly, perhaps in displacing elastin microassemblies from complexes with fibulin-5 and/or cell surface heparan sulfate proteoglycans.
23782690 Solid phase binding assays detected strong calcium-dependent binding of the short fibulins to immobilized heparin, suggesting that these fibulins may bind cell surface-located heparan sulfate
22829427 Data indicate mutations of FBLN4, FBLN5, and LTBP4 in 12 probands presenting with type 1 recessive cutis laxa.
22790990 fibulin-5 is reduced in patients with primary spontaneous pneumothorax who are younger than 25 years of age.
22685266 Altered expression of miR-200c may have a significant impact on the outcome of leiomyomas growth, maintenance of their mesenchymal and fibrotic characteristics, and possibly their associated symptoms.
22487196 Data suggest that expression of FBLN5 (fibulin 5) and LOXL1 (lysyl oxidase-like protein 1) (but not expression of elastin) is down-regulated in uterosacral ligaments of postmenopausal women with pelvic organ prolapse.
22280367 suggest that uPA promotes cell migration by binding to fibulin-5, initiating its cleavage by plasmin, which leads to its dissociation from beta1-integrin and thereby unblocks the capacity of integrin to facilitate cell motility
21576112 This study identifies fibulin-5 as a gene involved in Charcot-Marie-Tooth neuropathies and reveals heterozygous fibulin-5 mutations in 2% of our patients with age-related macular degeneration and hyperelastic skin.
21519142 MMP9 and fibulin-5 have roles in progression of pelvic organ prolapse in mice and humans
21454378 Role of fibulin-5 in metastatic organ colonization
21290250 spatiotemporal shift from the trophoblast compartment in first trimester to the villous vasculature at term suggests a dual role of fibulin-5 in human placental development.
21193390 HIF-1 signaling underlies the increase of FBLN5 expression elicited by hypoxia in endothelial cells suggesting that FBLN5 induction could be involved in the adaptive survival response of endothelial cells to hypoxia
21122382 The expression of EVEC in ovarian cancer and its metastatic sites was remarkably decreased.
20599731 Fibulin-5, a secreted extracellular matrix protein, was identified as a binding partner of Nogo-B.
20599547 Biophysical characterisation of fibulin-5 proteins associated with disease
20546612 Observational study of gene-disease association. (HuGE Navigator)
20379614 Clinical trial of gene-disease association and gene-environment interaction. (HuGE Navigator)
20201926 Observational study and genome-wide association study of gene-disease association. (HuGE Navigator)
20133342 genetic variation within FBLN5 is unlikely to play any role in the development of abdominal aortic aneurysm
20007835 The results identified structural differences for the disease-causing cutis laxa mutants and for one AMD (age-related macular degeneration) variant (G412E), suggesting that this may also be pathogenic.
19957551 The decreased expression of elastin and fibulin-5 was correlated with degree of pelvic organ prolapse.
19948975 Observational study of gene-disease association, gene-gene interaction, gene-environment interaction, and genetic testing. (HuGE Navigator)
19862539 FIB-5 expression is decreased in uterosacral ligaments of women with uterine prolapse.
19767220 Data show that the growth rate of the fibulin-5 transfected GFP-F5 cells was remarkably reduced than that of the untransfected cells.
19617354 fibulin 5 functions as a dimer during elastinogenesis or that dimerization may provide a method for limiting interactions with binding partners such as tropoelastin.
19570982 fibulin-5 controlled elastin deposition on microfibrils, although fibulin-4 can also bind fibrillin-1.
19450918 There was decreased expression of fibulin-5 and increased expression of lysyl oxidase-like 1 in uterosacral ligaments in patients with pelvic organ prolapse, which suggests the possibility of defects in elastin synthesis.
19251763 Results show substantially decreased gene signals for production of small leucine-rich repeat proteoglycans and fibulin-5 in pelvic organ prolapse.
19194475 homozygous recessive mutations in exon 12 of the elastin gene (p.P211S) in three patients from two related consanguineous Syrian families;a polymorphism in the fibulin 5 gene in one seems to modify the phenotype, producing more severe symptoms
19167375 Data revealed that fibulin-5 is able to separately interact with tropoelastin or fibrillin-1 in the culture medium. Moreover, elastin, fibrillin-1, and fibulin-5 co-localize in the extracellular matrix.
19015224 Observational study of gene-disease association, gene-environment interaction, and pharmacogenomic / toxicogenomic. (HuGE Navigator)
18989607 Fibulin-5 expression is decreased in vaginal biopsies from women with prolapse. Changes in fibulin expression may play a role in the development of pelvic organ prolapse.
18341572 fibulin-5 is a critical component in the control of elastic fibre assembly by dermal fibroblasts
18222970 degradation of basement membrane by cathepsin D liberates both fibulin-1 fragments and fibulin-5, which function to inhibit angiogenesis
18185537 the p.C217R mutation in fibulin-5 from cutis laxa patients is associated with incomplete extracellular matrix formation in a skin equivalent model
17581631 These results suggest a novel regulatory mechanism of elastic fiber assembly in which LTBP-2 regulates targeting of DANCE on suitable microfibrils to form elastic fibers.
17472576 This study identified the beta1 integrins on primary smooth muscle cells that fibulin-5 interacts with, and showed that failure of fibulin-5 to activate these receptors limits cell spreading, migration and proliferation.
17371835 Fibulin-5 protein potently induces elastic fiber assembly and maturation by organizing tropoelastin and cross-linking enzymes onto microfibrils.
17255108 ternary complex formation between fibrillin-1, fibulins, and tropoelastin demonstrated that fibulin-2 and -5 but much less fibulin-4, are able to act as molecular adaptors between fibrillin-1 and tropoelastin
17035250 Fibulin-5 is necessary for elastic fiber formation by facilitating the deposition of elastin onto a microfibrillar scaffold via direct molecular interactions.
16904068 Endothelial cell (EC) attachment and adhesion to an artificial surface is improved after fibulin-5 (FBLN5) gene transfer. With improved adhesion, EC transduced to express FBLN5 exhibits reduced proliferative rates.
16837650 These studies demonstrate that induction of fibulin 5 gene expression in lung fibroblasts is mediated via canonical TGF-beta/Smad signaling and requires the PI3-kinase/Akt pathway.
16374472 The interaction of elastin and fibulin-5 alleles results in elastic fibers susceptible to inflammatory destruction.
16153447 Decreased fibulin-5 may contribute to the pathogenesis of aortic dissection by impairing elastic fiber assembly.
15528465 EcSOD-fibulin-5 interaction is needed for ecSOD binding to vascular tissues, regulating their O2*- levels. This is a new mechanism for controlling vascular redox state in the extracellular space in cardiovascular diseases with high oxidative stress.
15269314 Observational study of gene-disease association. (HuGE Navigator)
15269314 Missense mutations in the fibulin 5 gene were found in 1.7 percent of patients with AMD.
12618961 Genetic heterogeneity of cutis laxa exists due to a heterozygous tandem duplication in the FBLN5 gene.
12111551 Direct interaction of the extracellular matrix protein DANCE with apolipoprotein(a) mediated by the kringle IV-type 2 domain.

AA Sequence

MPGIKRILTVTILALCLPSPGNAQAQCTNGFDLDRQSGQCLDIDECRTIPEACRGDMMCVNQNGGYLCIP      1 - 70
RTNPVYRGPYSNPYSTPYSGPYPAAAPPLSAPNYPTISRPLICRFGYQMDESNQCVDVDECATDSHQCNP     71 - 140
TQICINTEGGYTCSCTDGYWLLEGQCLDIDECRYGYCQQLCANVPGSYSCTCNPGFTLNEDGRSCQDVNE    141 - 210
CATENPCVQTCVNTYGSFICRCDPGYELEEDGVHCSDMDECSFSEFLCQHECVNQPGTYFCSCPPGYILL    211 - 280
DDNRSCQDINECEHRNHTCNLQQTCYNLQGGFKCIDPIRCEEPYLRISDNRCMCPAENPGCRDQPFTILY    281 - 350
RDMDVVSGRSVPADIFQMQATTRYPGAYYIFQIKSGNEGREFYMRQTGPISATLVMTRPIKGPREIQLDL    351 - 420
EMITVNTVINFRGSSVIRLRIYVSQYPF                                              421 - 448
//

Text Mined References (90)

PMID Year Title
26577699 2015 Fibulin-5 Blocks Microenvironmental ROS in Pancreatic Cancer.
26506560 2015 Fibulin-5 is upregulated in decidualized human endometrial stromal cells and promotes primary human extravillous trophoblast outgrowth.
26494967 2015 Effect of fibulin-5 on adhesion, migration and invasion of hepatocellular carcinoma cells via an integrin-dependent mechanism.
26251522 2016 Fibulin-5 is a tumour suppressor inhibiting cell migration and invasion in ovarian cancer.
26095157 2016 Fibulin-5 regulates keloid-derived fibroblast-like cells through integrin beta-1.
25909283 2015 Fibulin-5 inhibits Wnt/?-catenin signaling in lung cancer.
25891043 2015 Expression of fibulin-5 in the skin of patients with rectal prolapse.
25845228 [Diverse functions of fibulin-5 in tumors].
25807371 2015 Analysis of disease-associated protein expression using quantitative proteomics—fibulin-5 is expressed in association with hepatic fibrosis.
25792650 Gene expression levels of elastin and fibulin-5 according to differences between carotid plaque regions.
25684523 2015 Fibulin-5 is a prognostic marker that contributes to proliferation and invasion of human glioma cells.
25494879 2014 Fibulin-5 inhibits hepatocellular carcinoma cell migration and invasion by down-regulating matrix metalloproteinase-7 expression.
25429064 2015 Meta-analysis of genome-wide association studies of adult height in East Asians identifies 17 novel loci.
25118846 2014 Nuclear translocation of lysyl oxidase is promoted by interaction with transcription repressor p66?.
24990664 2015 A large lung gene expression study identifying fibulin-5 as a novel player in tissue repair in COPD.
24917111 2014 Fibulin-5 (FBLN5) gene polymorphism is associated with pelvic organ prolapse.
24386352 2013 Oncogenic fibulin-5 promotes nasopharyngeal carcinoma cell metastasis through the FLJ10540/AKT pathway and correlates with poor prognosis.
24275569 2014 An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.
24148803 2014 LTBP-2 competes with tropoelastin for binding to fibulin-5 and heparin, and is a negative modulator of elastinogenesis.
23782690 2013 Fibulin-3, -4, and -5 are highly susceptible to proteolysis, interact with cells and heparin, and form multimers.
23533145 2013 In-depth proteomic analyses of exosomes isolated from expressed prostatic secretions in urine.
23376485 2013 Proteomic analysis of podocyte exosome-enriched fraction from normal human urine.
23328402 2013 Czech family confirms the link between FBLN5 and Charcot-Marie-Tooth type 1 neuropathy.
22829427 2013 Comprehensive clinical and molecular analysis of 12 families with type 1 recessive cutis laxa.
22790990 2012 Fibulin-5 protein is reduced in the lung of patients with spontaneous pneumothorax who are under 25 years old.
22685266 2012 miR-200c is aberrantly expressed in leiomyomas in an ethnic-dependent manner and targets ZEBs, VEGFA, TIMP2, and FBLN5.
22487196 2012 Decreased expression of elastin, fibulin-5 and lysyl oxidase-like 1 in the uterosacral ligaments of postmenopausal women with pelvic organ prolapse.
22280367 2012 Fibulin-5 binds urokinase-type plasminogen activator and mediates urokinase-stimulated ?1-integrin-dependent cell migration.
21576112 2011 Fibulin-5 mutations link inherited neuropathies, age-related macular degeneration and hyperelastic skin.
21519142 2011 Extracellular matrix proteases contribute to progression of pelvic organ prolapse in mice and humans.
21454378 2011 Role of fibulin-5 in metastatic organ colonization.
21290250 2011 Fibulin-5 expression in the human placenta.
21193390 2011 Fibulin-5 is up-regulated by hypoxia in endothelial cells through a hypoxia-inducible factor-1 (HIF-1?)-dependent mechanism.
21122382 2010 [Expression of EVEC in ovarian carcinoma and its biological significance].
20599731 2010 Nogo-B mediates HeLa cell adhesion and motility through binding of Fibulin-5.
20599547 2010 Biophysical characterisation of fibulin-5 proteins associated with disease.
20551380 2010 Proteomics characterization of extracellular space components in the human aorta.
20546612 2010 The role of height-associated loci identified in genome wide association studies in the determination of pediatric stature.
20379614 Personalized smoking cessation: interactions between nicotine dose, dependence and quit-success genotype score.
20201926 2010 Human variation in alcohol response is influenced by variation in neuronal signaling genes.
20133342 2010 Common polymorphisms of Fibulin-5 and the risk of abdominal aortic aneurysm development.
20007835 2010 Structural effects of fibulin 5 missense mutations associated with age-related macular degeneration and cutis laxa.
19957551 2009 [Expression and significance of elastin and fibulin-5 in anterior vaginal tissue of women with pelvic organ prolapse].
19948975 2009 Integrative predictive model of coronary artery calcification in atherosclerosis.
19862539 2010 Differential expression of fibulins in the uterosacral ligaments of women with uterine prolapse.
19767220 Fibulin-5 is down-regulated in urothelial carcinoma of bladder and inhibits growth and invasion of human bladder cancer cell line 5637.
19617354 2009 Fibulin 5 forms a compact dimer in physiological solutions.
19570982 2009 Differential regulation of elastic fiber formation by fibulin-4 and -5.
19450918 2009 Changes in expression of fibulin-5 and lysyl oxidase-like 1 associated with pelvic organ prolapse.
19251763 2009 Gene expressions of small leucine-rich repeat proteoglycans and fibulin-5 are decreased in pelvic organ prolapse.
19194475 2009 An autosomal-recessive form of cutis laxa is due to homozygous elastin mutations, and the phenotype may be modified by a heterozygous fibulin 5 polymorphism.
19167375 2009 DANCE/fibulin-5 promotes elastic fiber formation in a tropoelastin isoform-dependent manner.
19015224 2009 Polymorphisms in C2, CFB and C3 are associated with progression to advanced age related macular degeneration associated with visual loss.
18989607 2009 Fibulin-5 expression is decreased in women with anterior vaginal wall prolapse.
18391951 2008 Many sequence variants affecting diversity of adult human height.
18341572 2008 Fibulin-5 accelerates elastic fibre assembly in human skin fibroblasts.
18222970 2008 Basement membrane derived fibulin-1 and fibulin-5 function as angiogenesis inhibitors and suppress tumor growth.
18185537 2008 A p.C217R mutation in fibulin-5 from cutis laxa patients is associated with incomplete extracellular matrix formation in a skin equivalent model.
18029348 2008 Toward a confocal subcellular atlas of the human proteome.
17581631 2007 Latent TGF-beta-binding protein 2 binds to DANCE/fibulin-5 and regulates elastic fiber assembly.
17472576 2007 Fibulin-5 binds human smooth-muscle cells through alpha5beta1 and alpha4beta1 integrins, but does not support receptor activation.
17371835 2007 Fibulin-5/DANCE has an elastogenic organizer activity that is abrogated by proteolytic cleavage in vivo.
17255108 2007 Fibrillin-1 interactions with fibulins depend on the first hybrid domain and provide an adaptor function to tropoelastin.
17035250 2006 Fibulin-5 mutations: mechanisms of impaired elastic fiber formation in recessive cutis laxa.
16904068 2006 Effects of fibulin-5 on attachment, adhesion, and proliferation of primary human endothelial cells.
16837650 2006 Fibulin-5 gene expression in human lung fibroblasts is regulated by TGF-beta and phosphatidylinositol 3-kinase activity.
16713569 2006 A protein-protein interaction network for human inherited ataxias and disorders of Purkinje cell degeneration.
16691202 2006 Homozygous missense mutation in fibulin-5 in an Iranian autosomal recessive cutis laxa pedigree and associated haplotype.
16652333 2006 Reduced secretion of fibulin 5 in age-related macular degeneration and cutis laxa.
16374472 2006 Inflammatory destruction of elastic fibers in acquired cutis laxa is associated with missense alleles in the elastin and fibulin-5 genes.
16344560 2006 Diversification of transcriptional modulation: large-scale identification and characterization of putative alternative promoters of human genes.
16303743 2005 Signal sequence and keyword trap in silico for selection of full-length human cDNAs encoding secretion or membrane proteins from oligo-capped cDNA libraries.
16153447 2005 Decreased expression of fibulin-5 correlates with reduced elastin in thoracic aortic dissection.
15790312 2005 Fibulin-5 interacts with fibrillin-1 molecules and microfibrils.
15528465 2004 Fibulin-5 is a novel binding protein for extracellular superoxide dismutase.
15489334 2004 The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
15342556 2004 Sequence comparison of human and mouse genes reveals a homologous block structure in the promoter regions.
15269314 2004 Missense variations in the fibulin 5 gene and age-related macular degeneration.
14745449 2004 Elastic fiber homeostasis requires lysyl oxidase-like 1 protein.
14702039 2004 Complete sequencing and characterization of 21,243 full-length human cDNAs.
12975309 2003 The secreted protein discovery initiative (SPDI), a large-scale effort to identify novel human secreted and transmembrane proteins: a bioinformatics assessment.
12618961 2003 Genetic heterogeneity of cutis laxa: a heterozygous tandem duplication within the fibulin-5 (FBLN5) gene.
12477932 2002 Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences.
12189163 2002 Homozygosity for a missense mutation in fibulin-5 (FBLN5) results in a severe form of cutis laxa.
12111551 2002 Direct interaction of the extracellular matrix protein DANCE with apolipoprotein(a) mediated by the kringle IV-type 2 domain.
11805835 2002 Fibulin-5/DANCE is essential for elastogenesis in vivo.
11805834 2002 Fibulin-5 is an elastin-binding protein essential for elastic fibre development in vivo.
11549288 2001 M phase-specific association of human topoisomerase IIIbeta with chromosomes.
10640802 1999 Assignment of fibulin-5 (FBLN5) to human chromosome 14q31 by in situ hybridization and radiation hybrid mapping.
10428823 1999 DANCE, a novel secreted RGD protein expressed in developing, atherosclerotic, and balloon-injured arteries.