| 產(chǎn)品編號 | bs-23735R-APC |
| 英文名稱(chēng) | Rabbit Anti-Fibulin 5/APC Conjugated antibody |
| 中文名稱(chēng) | APC標記的衰老關(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. |
| 規格價(jià)格 | 100ul/2980元 購買(mǎi) 大包裝/詢(xún)價(jià) |
| 說(shuō) 明 書(shū) | 100ul |
| 研究領(lǐng)域 | 心血管 免疫學(xué) 信號轉導 內分泌病 細胞骨架 細胞外基質(zhì) |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Polyclonal |
| 交叉反應 | (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, ) |
| 產(chǎn)品應用 | ICC=1:50-200 IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 48kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Fibulin 5 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲 存 液 | Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.05M PB, pH 7.5. |
| 保存條件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 產(chǎn)品介紹 |
background: 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. 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(chēng)為FBLN-5、DANCE或EVEC是細胞外基質(zhì)蛋白質(zhì)家族的一員，在組織器官發(fā)育、重塑和修復過(guò)程中起重要作用，并與內皮細胞相互作用.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合,并將后者錨于細胞表面,這對形成彈性纖維十分關(guān)鍵, 對血管的發(fā)育和修復具有重要作用.此外,Fibuljn-5還能促進(jìn)創(chuàng )口愈合, 與細胞的增殖、運動(dòng)和侵襲有關(guān) fibulin-5有學(xué)者稱(chēng)“皮膚衰老關(guān)鍵蛋白”與皮膚彈性有關(guān)的蛋白，對于起著(zhù)固定細胞外壁、保持肌膚緊繃、維護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)者認為：fibulin-5能夠抑制血管的形成，該蛋白質(zhì)在腫瘤轉移過(guò)程中表達降低或消失，將有可能用于腫瘤治療方面的研究。 |
