生物来源
mouse
质量水平
抗体形式
purified immunoglobulin
抗体产品类型
primary antibodies
克隆
689, monoclonal
种属反应性
mouse
种属反应性(根据同源性预测)
chicken (based on 100% sequence homology), human (based on 100% sequence homology)
技术
ELISA: suitable
immunohistochemistry: suitable
同位素/亚型
IgG1κ
NCBI登记号
UniProt登记号
运输
wet ice
靶向翻译后修饰
unmodified
基因信息
human ... EFEMP1(2202)
一般描述
Fibrillins are glycosolated and disulfide-rich components of the extracellular matrix belonging to the Fibrillin-LTBP protein family. They serve an important function in both nonelastic and elastic tissues such as eye, bone and blood vessels. The functional units of Fibrillin are known as microfibrils, multiprotein assemblies, which act as structural scaffolding for the limiting of the tissue elasticity, structural integrity of different organ systems, the assembly of elastic fibers, and regulation of BMP growth factor signaling. There are three known isoforms; Fibrillin 1, Fibrillin 2, and Fibrillin 3. Fibrillin 3 is the most recently described member of the Fibrillin family, with highest expression in the brain. Alterations in the microfibril assembly often occur in patients with fibrillinopathies such as; Congenital Contractual Arachnodactyly (CCA) which results in disruption of Fibrillin 2, and Marfan’s syndrome resulting from Fibrillin 1 mutation.
特异性
The antibody recognizes isoforms 1, 2, and 3 of fibrillin.
免疫原
Recombinant protein corresponding to human Fibrillin 3.
应用
Anti-Fibrillin (Pan) Antibody, clone 689 detects level of Fibrillin (Pan) & has been published & validated for use in IH, ELISA.
ELISA Analysis: This antibody has been used by an independent laboratory in ELISA (Corson, 2003).
质量
Evaluated by Immunohistochemistry in fresh frozen mouse skin tissue.
Immunohistochemistry Analysis: 1:200 dilution of the antibody detected Fibrillin (Pan) in fresh frozen mouse skin tissue.
Immunohistochemistry Analysis: 1:200 dilution of the antibody detected Fibrillin (Pan) in fresh frozen mouse skin tissue.
目标描述
~ 350 kDa calculated
外形
Format: Purified
分析说明
Control
Mouse skin tissue
Mouse skin tissue
其他说明
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
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储存分类代码
12 - Non Combustible Liquids
WGK
WGK 1
闪点(°F)
Not applicable
闪点(°C)
Not applicable
Yong Yuan et al.
PloS one, 14(3), e0212569-e0212569 (2019-03-07)
Pseudoexfoliation (PEX) syndrome is an age-related systemic disease with ocular manifestations. The development of animal models is critical in order to elucidate the cause of the disease and to test potential treatment regimens. The purpose of this study is to
Tomoko Matsuzaki et al.
Journal of cellular physiology, 236(3), 1980-1995 (2020-07-31)
Fibrillins (FBNs) form mesh-like structures of microfibrils in various elastic tissues. RECK and FBN1 are co-expressed in many human tissues, suggesting a functional relationship. We found that dermal FBN1 fibers show atypical morphology in mice with reduced RECK expression (RECK-Hypo
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