anti-Fibulin 4 (FBLN4) Antibodies

Human Fibulin 4 (FBLN4) interaction partners

1. different mutations in the fibulin-4 gene result in different molecular defects affecting secretion rates, protein stability, LOX-induced cross-linking, or binding to other ECM components and molecules of the TGF-beta pathway.

2. the expression of fibulin-4 was negatively correlated with the malignant phenotype of endometrial cancer cells. Fibulin-4 may have the ability to suppress endometrial cancer cell invasion and proliferation

3. The results revealed that fibulin-4 expression was upregulated in osteosarcoma, and was positively correlated with low differentiation, lymph node metastasis, and poor prognosis. Fibulin-4 was also found to be over-expressed in highly invasive cell lines and in the highly invasive subclones

4. link between Fibulin-4 and the canonical Wnt/beta-catenin pathway that may contribute to our understanding of the molecular mechanisms of OA

5. Findings suggested that fibulin-4 is important for the proteolytic activation of lysyl oxidase which has a pivotal role in cross-linking of collagen and elastin.

6. Data indicates that Fibulin-4 is a novel gene that is found overexpressed in ovarian cancer and associated with poor prognostic clinicopathologic features.

7. pulmonary emphysema in aneurysmal Fibulin-4 deficient (Fibulin-4(R)) mice

8. Homozygous mutations in exon 7 of the FBLN4 gene can produce lethal vasculopathy.

9. This study demonstrated that fibulin-4 may serve as a new prognostic factor and as a potential therapeutic target for patients with cervical carcinoma.

10. The expression level of EFEMP2 is dramatically increased in colorectal cancer patients, even at the early stage, compared with healthy controls.

11. Studied Fibulin-4 expression in aortic wall to find out its role in aortic dissection development. Used samples of aortic wall from 10 patients operated for acute ascending aortic dissection and five patients for chronic ascending aortic dissection.

12. 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

13. Data indicate mutations of FBLN4, FBLN5, and LTBP4 in 12 probands presenting with type 1 recessive cutis laxa.

14. A lethal, genetic disorder characterized by severe deformation of elastic arteries, was linked to novel mutations in the FBLN4 gene.

15. Low EFEMP2 expression is associated with malignant pleural mesothelioma.

16. EFEMP2 is a novel PITX2-interacting protein that may bear importance for the development of anterior segment dysgenesis (ASD) and glaucoma.

17. FBLN4 has 10 coding exons

18. In this largest cohort of reported patients with a mutated EFEMP2 gene, we illustrate the phenotypic spectrum of inherited AA due to a novel EFEMP2 mutation.

19. patients with recessive FBLN4 mutations are predominantly characterized by aortic aneurysms, arterial tortuosity and stenosis; evidence for the involvement of altered TGFbeta signaling in the pathogenesis of FBLN4 mutations

20. Fibulin-4, expressed in chondrocytes and recognized as an autoantigen mainly in osteoarthritis (OA) rather than in rheumatoid arthritis, may play pathogenic roles in OA.

Mouse (Murine) Fibulin 4 (FBLN4) interaction partners

1. These data clearly establish a functional link between Ltbp-4L and fibulin-4 as a crucial molecular requirement for survival and elastogenesis in mice.

2. fibulin-4 seems to play a role in regulating tendon collagen fibrillogenesis, in addition to its essential function in elastogenesis.

3. Findings suggested that fibulin-4 is important for the proteolytic activation of lysyl oxidase which has a pivotal role in cross-linking of collagen and elastin.

4. These data provide new insights in the molecular interaction between Fibulin-4 and TGF-beta pathway regulation in the pathogenesis of aortic aneurysms.

5. Fibulin-4 serves as a potential scaffolding protein during collagen maturation in the extracellular space and loss of fibulin-4 disrupts collagen synthesis and maturation.

6. fibulin-4 plays a role in regulating collagen fibril assembly and offer a preclinical platform for developing treatments for autosomal recessive cutis laxa 1B.

7. MBP1 inhibits Ab fibril formation in vitro and demonstrate the ability of MBP1 to reduce Ab pathology and improve behavioral performance.

8. pulmonary emphysema in aneurysmal Fibulin-4 deficient (Fibulin-4(R)) mice

9. Smooth muscle cell (SMC)-specific deletion of Fibulin-4 knockout ascending aorta and carotid artery showed mechanical changes in the axial direction.

10. Compared to control SMCs, the modulus of Eln-/- SMCs is reduced by 40%, but is unchanged in Fbln4-/- SMCs. The Eln-/- SMC modulus is rescued by soluble or alpha elastin treatment.

11. abnormal up-regulation of angiotensin-converting enzyme (ACE) in SMCs and subsequent activation of angiotensin II (AngII) signaling are involved in the onset of aortic aneurysms in Fbln4 mice.

12. Data show that fibulin-4 staining intensity in the ligamentum flavum was greatest at P14, though moderately strong at P7 and P21, and efore P7 and after P28, staining was detectable but relatively minimal.

13. Data show that the structural and functional alterations were accompanied by upregulation of TGF-beta signaling in aortas from fibulin-4 deficient mice.

14. Report coregistration of MMP activity by noninvasive tomographic imaging methods allows the detection of increased MMP activity, even before the aneurysm has actually formed in fibulin-4 deficient mice.

15. Fibulin-4 plays a dual role in the formation of elastic fibers as well as terminal differentiation and maturation of SMCs in the aortic wall.

16. fibulin-4 plays an irreplaceable role in elastogenesis.

17. Transcriptome and protein expression analysis implicate perturbation of TGF-beta signaling in the pathogenesis of aneurysm in fibulin-4 deficient mice.

18. A sufficient amount of FBLN4 is essential for tethering LOX to tropoelastin to facilitate cross-linking during elastogenesis.