FBLN1是一種與細(xì)胞外基質(zhì)相互作用的蛋白，已被研究與多種疾病的發(fā)病機(jī)制有關(guān)，如心血管疾病、癌癥、肝病等。近期研究發(fā)現(xiàn)FBLN1還參與了免疫炎癥反應(yīng)的調(diào)節(jié)，可以抑制單核細(xì)胞的活化和炎癥細(xì)胞的浸潤，為探索新的治療策略提供了新思路。

Incorporated into fibronectin-containing matrix fibers. May play a role in cell adhesion and migration along protein fibers within the extracellular matrix (ECM). Could be important for certain developmental processes and contribute to the supramolecular organization of ECM architecture, in particular to those of basement membranes. Has been implicated in a role in cellular transformation and tumor invasion, it appears to be a tumor suppressor. May play a role in haemostasis and thrombosis owing to its ability to bind fibrinogen and incorporate into clots. Could play a significant role in modulating the neurotrophic activities of APP, particularly soluble APP.

1. FBLN1 and STK31 can be possible screening and differentiating biomarkers of colorectal cancer PMID: 29715435
2. Fibulin-1 is expressed in breast cancer cell lines, interacts wtih SHBG, and is increased by estradiol PMID: 29940241
3. This study demonstrated that FBLN1 can effectively promote osteogenic differentiation of human nasal inferior turbinate-derived mesenchymal stem cells into osteoblasts. PMID: 28445604
4. This study demonstrated that fibulin-1 (FBLN1) was detected in the serums of Alzheimer disease patients PMID: 27911324
5. Interestingly and in contrast with our expectation, we found that the expression level of FBLN-4 and BCRP were downregulated in tumor compared to adjacent normal tissues. FBLN-4 was associated with grade histology and therefore can be considered as a potential prognostic biomarker. PMID: 28282800
6. Serum and vitreous fibulin-1 concentrations are elevated under diabetic retinopathy condition. PMID: 27422995
7. Galectin-3 and fibulin-1 levels are elevated in heart failure patients with impaired glucose metabolism. Reduced LV contractile reserve among HF patients with DM does not to have an independent impact on plasma Gal-3 and fibulin-1 levels. PMID: 28068900
8. changes in methylation levels in cfDNA associated with hepatocellular carcinoma and could represent useful plasma-based biomarker PMID: 28333958
9. The FBLN1/FGF8 interaction may also be involved in the survival of neural crest cell population during development. PMID: 27402846
10. Low expression of fibulin-1 is associated with gastric cancer. PMID: 26779638
11. LDH associated positively with fibulin-1 (beta = 0.23; p < 0.001) and ROS (beta = 0.11; p = 0.30) in blacks only with cardiovascular disease. PMID: 26631026
12. The procedure described is the first to our knowledge that enables a large scale purification of Fibulin-1 from human plasma. PMID: 26826315
13. Study identifies a novel fibulin-1D variant, named fibulin-1D', comprising 638 amino acids with molecular weight of 70.5 kDa. Fibulin-1D' lacks a secretion sequence, has a truncated N-terminus domain and binds to the intracellular domain of integrin beta1. PMID: 25661773
14. The purpose of this study was to develop and characterize robust quantitative assays for the emerging cardiovascular biomarker fibulin-1 and its circulating isoforms in human plasma. PMID: 25331251
15. Extracellular matrix biomarker, fibulin-1 and its association with soluble uPAR in a bi-ethnic South African population PMID: 25456503
16. Treatment with low-dose spironolactone reduced plasma fibulin-1 levels in patients with type 2 diabetes and resistant hypertension. PMID: 24739800
17. 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. PMID: 25234557
18. Plasma fibulin-1 is independently associated with pulse wave velocity in type 2 diabetics. PMID: 23866070
19. Increased levels of fibulin-1 were independently associated with higher levels of suPAR and NT-proBNP especially in patients with lower aortic valve stenosis PMID: 25014213
20. Mutation of fibulin-1 causes a novel syndrome involving the central nervous system and connective tissues. PMID: 24084572
21. FBLN1 functions as a novel candidate tumor suppressor gene in CM, and its downregulation may be due to promoter hypermethylation. PMID: 23907575
22. Increased plasma fibulin-1 levels are associated with diabetes and impaired kidney function. PMID: 23294625
23. Fibulin-1 is down regulated in renal cell carcinoma through promoter hypermethylation. PMID: 23391467
24. In single regression analysis, NT-proBNP was significantly associated with fibulin-1 in African men and Caucasian women. PMID: 22349089
25. Fibulin-1 is down-regulated in atrial tissue from patients with atrial fibrillation, perhaps reflecting the myocardial structural changes that may have taken place during arrhythmia. PMID: 20451270
26. plasma levels of fibulin-1 could serve as a potential indicator to monitor kidney malfunction or kidney damage PMID: 21888404
27. 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. PMID: 21926180
28. Promoter hypermethylation of FBLN1 was significantly associated with advanced stage hepatocellular carcinoma, multiple tumors and increased tumor size. PMID: 21268132
29. data collectively suggest fibulin-1C may be worthy of further investigation as a target for airway remodeling in asthma PMID: 20967215
30. Fibulin-1 protein was expressed in guinea pig sclera and cultured human scleral fibroblasts. PMID: 20405022
31. the pattern of fibulin-1 within human atherosclerotic lesions and the potential for fibulin-1, perhaps derived from the blood and acting in conjunction with fibrinogen, to play a role in the etiology and cardiovascular disease progression PMID: 19693531
32. Fibulin-1 has pronounced inhibitory effects on cell attachment and spreading promoted by fibronectin (FN) and suppresses the motility of a variety of cell types on FN substrates. PMID: 11792823
33. structural and functional characterization of promoter PMID: 11829738
34. FBLN1 disrupted by a t(12;22) translocation is associated with a complex type of synpolydactyly. PMID: 11836357
35. binding of HPV E6 protein to fibulin-1 PMID: 12200142
36. findings indicate that elevated expression and altered processing of fibulin-1 is associated with human breast cancer PMID: 12644824
37. The gene responsible for a unique vitreoretinal dystrophy is located on chromosome 22q13. PMID: 12912698
38. mutations in the splice acceptor site of fibulin-1 exon 19, but no MYH9 mutations,were found in family individuals affected by autosomal-dominant giant platelet syndromes associated with aberrant antisense gene regulation of the fibulin-1 gene. PMID: 14635206
39. FBLN seems to have an immunological and pathobiological role in breast neoplasm surveillance. PMID: 14691454
40. The preferential induction of the fibulin-1C variant, which is overexpressed in ovarian and breast cancer, might play an important role in estrogen-promoted carcinogenesis. PMID: 15528301
41. fiblin-1 is an important molecule that mediates progesterone action in human endometrial stromal cells differentiation towards implantation PMID: 15774544
42. fibulin-1 is a new regulator of ADAMTS-1-mediated proteoglycan proteolysis and may play an important role in proteoglycan turnover in tissues where there is overlapping expression PMID: 16061471
43. Coexpression of fibulin-1 with GROalpha abrogates key aspects of the transformed phenotype, including colonic tumor formation in a murine xenograft model. PMID: 17062666
44. degradation of basement membrane by cathepsin D liberates both fibulin-1 fragments and fibulin-5, which function to inhibit angiogenesis PMID: 18222970
45. FBLN1 was identified as a novel candidate tmour suppressor gene epigenetically downregulated in gastric cancer PMID: 18985039
46. Like tenascin-C, fibulin-1 inhibits fibroblast spreading and cell-mediated contraction of a fibrin-fibronectin matrix. PMID: 19109427
47. fibulin-1 and -2 respond differentially to single and repeated damaging noxae, and their expression is differently present in liver cells PMID: 19609566

顯示更多

收起更多

A chromosomal aberration involving FBLN1 is found in a complex type of synpolydactyly referred to as 3/3-prime/4 synpolydactyly associated with metacarpal and metatarsal synostoses. Reciprocal translocation t(12;22)(p11.2;q13.3) with RASSF8. Fibroblasts derived from a patient with synpolydactyly displayed alterations in the level of isoform D splice variant incorporated into the ECM and secreted into the conditioned culture medium. By contrast, the expression of isoform C was not perturbed in the patients fibroblasts. Furthermore, no aberrant polypeptides were detected in extracts of cultured patients fibroblasts. The translocation t(12;22) may result in haploinsufficiency of the isoform D splice variant, which could lead to the observed limb malformation.

Secreted, extracellular space, extracellular matrix.

Fibulin family

Isoform A and isoform B are only expressed in placenta. Isoform C and isoform D are expressed in a variety of tissues and cultured cells.

HGNC: 3600

OMIM: 135820

KEGG: hsa:2192

STRING: 9606.ENSP00000331544

UniGene: Hs.24601