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品种反应性 主要应用 宿主 格式 抗体类型 Rhesus Macaque, M, H, R, Chp WB Rb Affinity Purified Polyclonal Antibody 描述 产品目录编号 AB6065 描述 Anti-Fibulin-5 Antibody Alternate Names - UP50
- Fibulin-5
- FIBL-5
- Developmental arteries and neural crest EGF-like protein
- Dance
- Urine p50 protein
背景信息 Fibulin-5 (also known as EVEC/DANCE) is an extracellular matrix protein. Fibulin-5 is most commonly found in blood vessels and cardiac valves during embryogenesis and in adult tissue that contains elastic fibers like the skin, lungs, uterus and aorta. Fibulin-5 travels to the surface of the elastic fibers and its activity is dependent on calcium. Fibulin-5 seems to work as a scaffold protein that facilitates the binding of the elastic fibers to cells and the formation of the elastic fibers themselves. Mutations of fibulin-5 have been associated with age-related macular degeneration (AMD) and cutis laxa (CL). 产品信息 格式 Affinity Purified 控制 - Mouse heart tissue lysate
演示 Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. 应用 应用 Anti-Fibulin-5 Antibody detects level of Fibulin-5 & has been published & validated for use in WB. 主要应用 - Western Blotting
生物信息 免疫原品种 KLH-conjugated linear peptide corresponding to human Fibulin-5 near the C-terminus. 表位 Near C-terminus 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Rabbit 特异性 This antibody recognizes Fibulin-5 near the C-terminus. 品种反应性 Rhesus MacaqueMouseHumanRatChimpanzee Species Reactivity Note Demonstrated to react with Mouse, Human, and Rat.
Predicted to react with Chimpanzee and Rhesus Macaque based on 100% sequence homology.抗体类型 Polyclonal Antibody Entrez基因编号 - NP_006320
Entrez基因汇总 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]. 基因符号 - DANCE
- FBLN5
纯化方法 Affinity Purified UniProt编号 - Q9UBX5
UniProt汇总 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 STRUCTURE: 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.
INVOLVEMENT IN DISEASE: Defects in FBLN5 are a cause of autosomal dominant cutis laxa (ADCL) [MIM:123700]. Hereditary cutis laxa refers to a heterogeneous group of connective tissue disorders characterized by cutaneous abnormalities and variable systemic manifestations. The most constant clinical feature is loose skin, sagging over the face and trunk. Hereditary cutis laxa is inherited in both autosomal dominant and autosomal recessive modes. Autosomal dominant cutis laxa is a relatively benign inherited and acquired connective tissue disorder.
Defects in FBLN5 are a cause of cutis laxa autosomal recessive type 1 (ARCL1) [MIM:219100]. Hereditary cutis laxa refers to a heterogeneous group of connective tissue disorders characterized by cutaneous abnormalities and variable systemic manifestations. The most constant clinical feature is loose skin, sagging over the face and trunk. Hereditary cutis laxa is inherited in both autosomal dominant and autosomal recessive modes. ARCL1 shows the most severe phenotype and has the poorest prognosis. In addition to the skin, internal organs enriched in elastic fibers, such as the lung and arteries, are affected. Ref.7 Ref.10
Defects in FBLN5 are the cause of age-related macular degeneration type 3 (ARMD3) [MIM:608895]. ARMD is a multifactorial 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 (known as drusen) that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Ref.9
SEQUENCE SIMILARITIES: Belongs to the fibulin family.
Contains 6 EGF-like domains.产品使用声明 质量保证 Evaluated by Western Blot in mouse heart tissue lysate.
Western Blot Analysis: 0.1 µg/mL of this antibody detected Fibulin-5 in 10 µg of mouse heart tissue lysate.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt. 包装信息 数量 100 µg
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