FBLN5 Antibody
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中文名稱：FBLN5兔多克隆抗體
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貨號：CSB-PA008454GA01HU
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規格：￥3,900
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其他：
產品詳情
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Uniprot No.：
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基因名：
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別名：ADCL2 antibody; ARCL1A antibody; ARMD3 antibody; Dance antibody; Developmental arteries and neural crest EGF like protein antibody; Developmental arteries and neural crest EGF-like protein antibody; EVEC antibody; Fbln5 antibody; FBLN5_HUMAN antibody; FIBL 5 antibody; FIBL-5 antibody; Fibulin-5 antibody; FLJ90059 antibody; UP50 antibody; Urine p50 protein antibody
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宿主：Rabbit
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反應種屬：Human,Mouse,Rat
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免疫原：Human FBLN5
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免疫原種屬：Homo sapiens (Human)
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抗體亞型：IgG
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純化方式：Antigen Affinity Purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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產品提供形式：Liquid
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應用范圍：ELISA,WB,IHC
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Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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功能：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.
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基因功能參考文獻：
- 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
- The results showed a significant upregulation of ECM1 and ITGB3, and a significant downregulation for FBLN5 in pelvic organ prolapse patients. PMID: 29729708
- Serum fibulin-5 concentration can be regarded as a biomarker for evaluating disease severity and predicting prognosis in intracerebral hemorrhage patients. PMID: 27106135
- Fibulin-5 expression is increased in the induced sputum of asthmatic patients. PMID: 28942149
- 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
- FBLN5 downregulation in human abdominal aortic aneurysm could contribute to extracellular matrix remodeling induced by the inflammatory component of the disease. PMID: 27692634
- Identify a SOX9/HDAC-dependent mechanism involved in the down-regulation of FBLN5 by inflammation in abdominal aortic aneurysms. PMID: 27089918
- 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
- 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
- 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
- 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
- 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
- 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
- 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
- Fibulin-5 may be implicated in the aetiology of rectal prolapse in a subgroup of young male patients. PMID: 25891043
- Fibulin-5 expression is a disease marker of hepatic fibrosis. PMID: 25807371
- Loss of FBLN5 expression is associated with glioma. PMID: 25684523
- 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
- 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
- These results indicate that Fibulin-5 inhibits hepatocellular carcinoma invasion and metastasis by suppressing matrix metalloproteinase-7 expression. PMID: 25494879
- 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
- 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
- We identified a clear gene signature for elastogenesis in COPD and propose FBLN5 as a novel player in tissue repair in COPD. PMID: 24990664
- 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
- fibulin-5 overexpression in NPC specimens correlated with advanced tumor metastasis indicating a poor 5-year overall survival PMID: 24386352
- 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
- Data indicate mutations of FBLN4, FBLN5, and LTBP4 in 12 probands presenting with type 1 recessive cutis laxa. PMID: 22829427
- fibulin-5 is reduced in patients with primary spontaneous pneumothorax who are younger than 25 years of age. PMID: 22790990
- 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
- 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
- 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
- The expression of EVEC in ovarian cancer and its metastatic sites was remarkably decreased. PMID: 21122382
- The decreased expression of elastin and fibulin-5 was correlated with degree of pelvic organ prolapse. PMID: 19957551
- Role of fibulin-5 in metastatic organ colonization PMID: 21454378
- 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
- MMP9 and fibulin-5 have roles in progression of pelvic organ prolapse in mice and humans PMID: 21519142
- 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
- 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
- FIB-5 expression is decreased in uterosacral ligaments of women with uterine prolapse. PMID: 19862539
- genetic variation within FBLN5 is unlikely to play any role in the development of abdominal aortic aneurysm PMID: 20133342
- Biophysical characterisation of fibulin-5 proteins associated with disease PMID: 20599547
- Fibulin-5, a secreted extracellular matrix protein, was identified as a binding partner of Nogo-B. PMID: 20599731
- 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
- Direct interaction of the extracellular matrix protein DANCE with apolipoprotein(a) mediated by the kringle IV-type 2 domain. PMID: 12111551
- Genetic heterogeneity of cutis laxa exists due to a heterozygous tandem duplication in the FBLN5 gene. PMID: 12618961
- Missense mutations in the fibulin 5 gene were found in 1.7 percent of patients with AMD. PMID: 15269314
- 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
- Decreased fibulin-5 may contribute to the pathogenesis of aortic dissection by impairing elastic fiber assembly. PMID: 16153447
- The interaction of elastin and fibulin-5 alleles results in elastic fibers susceptible to inflammatory destruction. PMID: 16374472
- 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
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相關疾病：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)
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亞細胞定位：Secreted. Secreted, extracellular space, extracellular matrix.
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蛋白家族：Fibulin family
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組織特異性：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
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