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Merck
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主要文件

MAB2641

Sigma-Aldrich

Anti-Fibrillin (Pan) Antibody, clone 689

clone 689, from mouse

别名:

EGF-containing fibulin-like extracellular matrix protein 1, Fibrillin-like protein, Extracellular protein S1-5

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About This Item

UNSPSC代码:
12352203
eCl@ss:
32160702
NACRES:
NA.41

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.

目标描述

~ 350 kDa calculated

外形

Format: Purified

分析说明

Control
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


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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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