| Tbio | Fibulin-5 |
Essential for elastic fiber formation, is involved in the assembly of continuous elastin (ELN) polymer and promotes the interaction of microfibrils and ELN (PubMed:18185537). Stabilizes and organizes elastic fibers in the skin, lung and vasculature (By similarity). 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 (PubMed:10428823). May act as an adapter that mediates the interaction between FBN1 and ELN (PubMed:17255108).
The protein encoded by this gene is a secreted, extracellular matrix protein containing an Arg-Gly-Asp (RGD) motif and calcium-binding EGF-like domains. It promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. It is prominently expressed in developing arteries but less so in adult vessels. However, its expression is reinduced in balloon-injured vessels and atherosclerotic lesions, notably in intimal vascular smooth muscle cells and endothelial cells. Therefore, the protein encoded by this gene may play a role in vascular development and remodeling. Defects in this gene are a cause of autosomal dominant cutis laxa, autosomal recessive cutis laxa type I (CL type I), and age-related macular degeneration type 3 (ARMD3). [provided by RefSeq, Jul 2008]
The protein encoded by this gene is a secreted, extracellular matrix protein containing an Arg-Gly-Asp (RGD) motif and calcium-binding EGF-like domains. It promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. It is prominently expressed in developing arteries but less so in adult vessels. However, its expression is reinduced in balloon-injured vessels and atherosclerotic lesions, notably in intimal vascular smooth muscle cells and endothelial cells. Therefore, the protein encoded by this gene may play a role in vascular development and remodeling. Defects in this gene are a cause of autosomal dominant cutis laxa, autosomal recessive cutis laxa type I (CL type I), and age-related macular degeneration type 3 (ARMD3). [provided by RefSeq, Jul 2008]
Comments
| Disease | Target Count | Z-score | Confidence |
|---|---|---|---|
| Cutis laxa | 28 | 6.603 | 3.3 |
| Disease | Target Count | Z-score | Confidence |
|---|---|---|---|
| Age related macular degeneration | 84 | 3.732 | 1.9 |
| Charcot-Marie-Tooth disease | 74 | 0.0 | 4.0 |
| Neuropathy | 210 | 0.0 | 4.0 |
| Disease | Target Count | Z-score | Confidence |
|---|---|---|---|
| Tricuspid valve prolapse | 1 | 3.689 | 1.8 |
| Retinal drusen | 5 | 3.226 | 1.6 |
| Rectal prolapse | 12 | 3.143 | 1.6 |
| Disease | log2 FC | p |
|---|---|---|
| malignant mesothelioma | -5.400 | 0.000 |
| psoriasis | -1.700 | 0.002 |
| posterior fossa group B ependymoma | 2.500 | 0.000 |
| Atopic dermatitis | -1.500 | 0.002 |
| adrenocortical carcinoma | -2.697 | 0.000 |
| tuberculosis and treatment for 6 months | -1.500 | 0.000 |
| non-small cell lung cancer | -2.369 | 0.000 |
| intraductal papillary-mucinous adenoma (... | -2.500 | 0.000 |
| intraductal papillary-mucinous carcinoma... | -2.700 | 0.000 |
| intraductal papillary-mucinous neoplasm ... | -3.000 | 0.003 |
| colon cancer | -1.900 | 0.022 |
| lung cancer | -3.300 | 0.000 |
| breast carcinoma | -1.900 | 0.000 |
| lung adenocarcinoma | -2.200 | 0.000 |
| pediatric high grade glioma | 1.100 | 0.008 |
| pilocytic astrocytoma | 1.700 | 0.000 |
| sonic hedgehog group medulloblastoma | 1.300 | 0.001 |
| invasive ductal carcinoma | -2.700 | 0.000 |
| non-inflammatory breast cancer | -1.500 | 0.002 |
| lung carcinoma | -2.200 | 0.000 |
| ductal carcinoma in situ | -1.900 | 0.000 |
| pituitary cancer | -1.800 | 0.001 |
| Species | Source |
|---|---|
| Macaque | OMA Inparanoid |
| Mouse | OMA Inparanoid |
| Rat | OMA Inparanoid |
| Dog | OMA Inparanoid |
| Horse | OMA EggNOG Inparanoid |
| Cow | OMA Inparanoid |
| Platypus | OMA Inparanoid |
| Chicken | OMA Inparanoid |
| Anole lizard | OMA Inparanoid |
| Xenopus | OMA Inparanoid |
| Zebrafish | OMA Inparanoid |
| 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 |
| More... |
MPGIKRILTVTILALCLPSPGNAQAQCTNGFDLDRQSGQCLDIDECRTIPEACRGDMMCVNQNGGYLCIP 1 - 70 RTNPVYRGPYSNPYSTPYSGPYPAAAPPLSAPNYPTISRPLICRFGYQMDESNQCVDVDECATDSHQCNP 71 - 140 TQICINTEGGYTCSCTDGYWLLEGQCLDIDECRYGYCQQLCANVPGSYSCTCNPGFTLNEDGRSCQDVNE 141 - 210 CATENPCVQTCVNTYGSFICRCDPGYELEEDGVHCSDMDECSFSEFLCQHECVNQPGTYFCSCPPGYILL 211 - 280 DDNRSCQDINECEHRNHTCNLQQTCYNLQGGFKCIDPIRCEEPYLRISDNRCMCPAENPGCRDQPFTILY 281 - 350 RDMDVVSGRSVPADIFQMQATTRYPGAYYIFQIKSGNEGREFYMRQTGPISATLVMTRPIKGPREIQLDL 351 - 420 EMITVNTVINFRGSSVIRLRIYVSQYPF 421 - 448 //
| 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. | |
| More... |
