Human Fibulin-5(FBLN5) ELISA kit

Essential for elastic fiber formation, is involved in the assembly of continuous elastin (ELN) polymer and promotes the interaction of microfibrils and ELN. Stabilizes and organizes elastic fibers in the skin, lung and vasculature. Promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. Vascular ligand for integrin receptors which may play a role in vascular development and remodeling. May act as an adapter that mediates the interaction between FBN1 and ELN.

1. study reports the first Chinese family with c.1117C>T mutation of FBLN5; findings confirm a novel subtype of autosomal-dominant Charcot-Marie-Tooth disease due to a mutation in the FBLN5 gene PMID: 28332470
2. The results showed a significant upregulation of ECM1 and ITGB3, and a significant downregulation for FBLN5 in pelvic organ prolapse patients. PMID: 29729708
3. Serum fibulin-5 concentration can be regarded as a biomarker for evaluating disease severity and predicting prognosis in intracerebral hemorrhage patients. PMID: 27106135
4. Fibulin-5 expression is increased in the induced sputum of asthmatic patients. PMID: 28942149
5. Data show that fibulin-5 strongly binds to the endothelial cell surface reducing endothelial cell viability and interfering with the signaling pathways of the Ang-1/TIE-2 receptor axis. PMID: 27304216
6. FBLN5 downregulation in human abdominal aortic aneurysm could contribute to extracellular matrix remodeling induced by the inflammatory component of the disease. PMID: 27692634
7. Identify a SOX9/HDAC-dependent mechanism involved in the down-regulation of FBLN5 by inflammation in abdominal aortic aneurysms. PMID: 27089918
8. We also found that Fibulin-5 reduces the level of expression of Ki-67, a nuclear protein associated with cell proliferation. Moreover, reduction in Fibulin-5 expression corresponds to an increase of Ki-67 detection in breast tissue samples PMID: 26891749
9. 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 PMID: 26095157
10. Lower FBLN-5 expression is an important indicator of poor survival in hepatocellular carcinoma. FBLN-5 inhibits HCC adhesion/motility via integrin-dependent mechanism. PMID: 26494967
11. FBLN5 mRNA expression is upregulated in response to cAMP-mediated decidualization of primary human endometrial stromal cells, although FBLN5 itself does not enhance decidualization PMID: 26506560
12. 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. PMID: 26251522
13. 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. PMID: 26577699
14. Data suggest that fibulin-5 functions as a metastasis suppressor in lung cancer by modulating tumor microenvironment to suppress Wnt/beta-catenin signaling. PMID: 25909283
15. Fibulin-5 may be implicated in the aetiology of rectal prolapse in a subgroup of young male patients. PMID: 25891043
16. Fibulin-5 expression is a disease marker of hepatic fibrosis. PMID: 25807371
17. Loss of FBLN5 expression is associated with glioma. PMID: 25684523
18. Up-regulation of elastin and fibulin-5 mRNA levels in ICA were strongly correlated with family history of cardiovascular disease when compared to CCA PMID: 25792650
19. 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. PMID: 22487196
20. These results indicate that Fibulin-5 inhibits hepatocellular carcinoma invasion and metastasis by suppressing matrix metalloproteinase-7 expression. PMID: 25494879
21. 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) PMID: 25845228
22. 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. PMID: 24917111
23. We identified a clear gene signature for elastogenesis in COPD and propose FBLN5 as a novel player in tissue repair in COPD. PMID: 24990664
24. 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. PMID: 24148803
25. fibulin-5 overexpression in NPC specimens correlated with advanced tumor metastasis indicating a poor 5-year overall survival PMID: 24386352
26. 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 PMID: 23782690
27. Data indicate mutations of FBLN4, FBLN5, and LTBP4 in 12 probands presenting with type 1 recessive cutis laxa. PMID: 22829427
28. fibulin-5 is reduced in patients with primary spontaneous pneumothorax who are younger than 25 years of age. PMID: 22790990
29. 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. PMID: 22685266
30. 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 PMID: 22280367
31. Data show that the growth rate of the fibulin-5 transfected GFP-F5 cells was remarkably reduced than that of the untransfected cells. PMID: 19767220
32. The expression of EVEC in ovarian cancer and its metastatic sites was remarkably decreased. PMID: 21122382
33. The decreased expression of elastin and fibulin-5 was correlated with degree of pelvic organ prolapse. PMID: 19957551
34. Role of fibulin-5 in metastatic organ colonization PMID: 21454378
35. 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. PMID: 21576112
36. MMP9 and fibulin-5 have roles in progression of pelvic organ prolapse in mice and humans PMID: 21519142
37. 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. PMID: 21290250
38. 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 PMID: 21193390
39. FIB-5 expression is decreased in uterosacral ligaments of women with uterine prolapse. PMID: 19862539
40. genetic variation within FBLN5 is unlikely to play any role in the development of abdominal aortic aneurysm PMID: 20133342
41. Biophysical characterisation of fibulin-5 proteins associated with disease PMID: 20599547
42. Fibulin-5, a secreted extracellular matrix protein, was identified as a binding partner of Nogo-B. PMID: 20599731
43. 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. PMID: 20007835
44. Direct interaction of the extracellular matrix protein DANCE with apolipoprotein(a) mediated by the kringle IV-type 2 domain. PMID: 12111551
45. Genetic heterogeneity of cutis laxa exists due to a heterozygous tandem duplication in the FBLN5 gene. PMID: 12618961
46. Missense mutations in the fibulin 5 gene were found in 1.7 percent of patients with AMD. PMID: 15269314
47. 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. PMID: 15528465
48. Decreased fibulin-5 may contribute to the pathogenesis of aortic dissection by impairing elastic fiber assembly. PMID: 16153447
49. The interaction of elastin and fibulin-5 alleles results in elastic fibers susceptible to inflammatory destruction. PMID: 16374472
50. 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. PMID: 16837650

显示更多

收起更多

Neuropathy, hereditary, with or without age-related macular degeneration (HNARMD); Cutis laxa, autosomal dominant, 2 (ADCL2); Cutis laxa, autosomal recessive, 1A (ARCL1A); Macular degeneration, age-related, 3 (ARMD3)

Secreted. Secreted, extracellular space, extracellular matrix.

Fibulin family

Expressed in skin fibroblasts (at protein level)(PubMed:17035250). Expressed predominantly in heart, ovary, and colon but also in kidney, pancreas, testis, lung and placenta. Not detectable in brain, liver, thymus, prostate, or peripheral blood leukocytes

HGNC: 3602

OMIM: 219100

KEGG: hsa:10516

STRING: 9606.ENSP00000345008

UniGene: Hs.332708