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BN40219R-100ul
100ul
￥2360.00
交叉反應(yīng)：Human(predicted:Mouse,Rat,Cow) 推薦應(yīng)用：WB,ELISA
BN40219R-200ul
200ul
￥3490.00
交叉反應(yīng)：Human(predicted:Mouse,Rat,Cow) 推薦應(yīng)用：WB,ELISA
產(chǎn)品描述
| 英文名稱 | Fibulin 5 |
| 中文名稱 | 衰老關(guān)鍵蛋白抗體 |
| 別 名 | ARMD3; Dance; Developmental arteries and neural crest EGF like protein; FBLN5; FIBL 5; Developmental arteries and neural crest EGF-like protein; EVEC; Fbln5; FBLN5_HUMAN; UP50; FIBL 5; FIBL-5; Fibulin-5; FLJ90059; Urine p50 protein. |
| 研究領(lǐng)域 | 心血管 免疫學(xué) 信號(hào)轉(zhuǎn)導(dǎo) 內(nèi)分泌病 細(xì)胞骨架 細(xì)胞外基質(zhì) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Human, (predicted: Mouse, Rat, Cow, ) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000 ELISA=1:5000-10000 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 48kDa |
| 細(xì)胞定位 | 分泌型蛋白 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Fibulin 5:101-200/448 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| PubMed | PubMed |
| 產(chǎn)品介紹 | Fibulin 5: A protein that belongs to a family of extracellular proteins expressed in the basement membranes of blood vessels. Fibulin 5 may be essential for the polymerization of elastin. Missense mutations in FBLN5, the gene that encodes fibulin 5, appear responsible for 1-2% of cases of age-related macular degeneration (AMD). FBLN5 is located on chromosome 14 in band 14q32.1. See also: Fibulin 3. May play a role in vascular growth and maturation during development and in lesions of injured vessels. Function: Promotes adhesion of endothelial cells through interaction of integrins and the RGD motif. Could be a vascular ligand for integrin receptors and may play a role in vascular development and remodeling. Subunit: Homodimer. Subcellular Location: Secreted. Tissue Specificity: 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. DISEASE: Cutis laxa, autosomal dominant, 2 (ADCL2) [MIM:614434]: A connective tissue disorder characterized by loose, hyperextensible skin with decreased resilience and elasticity leading to a premature aged appearance. Face, hands, feet, joints, and torso may be differentially affected. Additional variable clinical features are gastrointestinal diverticula, hernia, and genital prolapse. Rare manifestations are pulmonary artery stenosis, aortic aneurysm, bronchiectasis, and emphysema. Note=The disease is caused by mutations affecting the gene represented in this entry. Cutis laxa, autosomal recessive, 1A (ARCL1A) [MIM:219100]: A connective tissue disorder characterized by loose, hyperextensible skin with decreased resilience and elasticity leading to a premature aged appearance. Face, hands, feet, joints, and torso may be differentially affected. The clinical spectrum of autosomal recessive cutis laxa is highly heterogeneous with respect to organ involvement and severity. Type I autosomal recessive cutis laxa is a specific, life-threatening disorder with organ involvement, lung atelectasis and emphysema, diverticula of the gastrointestinal and genitourinary systems, and vascular anomalies. Associated cranial anomalies, late closure of the fontanel, joint laxity, hip dislocation, and inguinal hernia have been observed but are uncommon. Note=The disease is caused by mutations affecting the gene represented in this entry. Age-related macular degeneration 3 (ARMD3) [MIM:608895]: A form of age-related macular degeneration, a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Note=Disease susceptibility is associated with variations affecting the gene represented in this entry. Similarity: Belongs to the fibulin family. Contains 6 EGF-like domains. SWISS: Q9UBX5 Gene ID: 10516 Database links: Entrez Gene: 10516 Human Entrez Gene: 23876 Mouse Omim: 604580 Human SwissProt: Q9UBX5 Human SwissProt: Q9WVH9 Mouse Unigene: 332708 Human Unigene: 288381 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. Fibulin-5 Fibulin-5亦稱為FBLN-5、DANCE或EVEC是細(xì)胞外基質(zhì)蛋白質(zhì)家族的一員，在組織器官發(fā)育、重塑和修復(fù)過程中起重要作用，并與內(nèi)皮細(xì)胞相互作用.Fibulin-5廣泛分布于富含彈性蛋白的組織, 能直接與原彈性蛋白結(jié)合,并將后者錨于細(xì)胞表面,這對形成彈性纖維十分關(guān)鍵, 對血管的發(fā)育和修復(fù)具有重要作用.此外,Fibuljn-5還能促進(jìn)創(chuàng)口愈合, 與細(xì)胞的增殖、運(yùn)動(dòng)和侵襲有關(guān) fibulin-5有學(xué)者稱“皮膚衰老關(guān)鍵蛋白”與皮膚彈性有關(guān)的蛋白，對于起著固定細(xì)胞外壁、保持肌膚緊繃、維護(hù)肺部和血管柔韌性作用的彈性纖維的發(fā)育十分關(guān)鍵. 還有學(xué)者認(rèn)為：fibulin-5能夠抑制血管的形成，該蛋白質(zhì)在腫瘤轉(zhuǎn)移過程中表達(dá)降低或消失，將有可能用于腫瘤治療方面的研究。 |
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