Anti-Fibrillin 1抗体This gene encodes a member of the fibrillin family. The encoded protein is a large, extracellular matrix glycoprotein that serve

产品编号 yb-1157R
英文名称Anti-Fibrillin 1抗体
中文名称 原纤维蛋白1抗体
别    名 fibrillin1; 350 kDa glycoprotein component extracellular microfibril; FBN 1; FBN1; FBN; Fibrillin 15; Fibrillin15; Marfan syndrome; MASS; MFS 1; MFS1; OCTD; SGS; Weill Marchesani syndrome; WMS; AI536462; B430209H23; Fib-1; Fibrilin-1; ACMICD; FBN1_HUMAN; GPHYSD2; SSKS; Weill Marchesani syndrome; WMS2.
Anti-Fibrillin 1抗体  
说 明 书 0.1ml  0.2ml  
研究领域 免疫学  信号转导  细胞骨架  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, 
产品应用 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 316kDa
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human FBN1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.

PubMed PubMed
产品介绍 background:
This gene encodes a member of the fibrillin family. The encoded protein is a large, extracellular matrix glycoprotein that serve as a structural component of 10-12 nm calcium-binding microfibrils. These microfibrils provide force bearing structural support in elastic and nonelastic connective tissue throughout the body. Mutations in this gene are associated with Marfan syndrome, isolated ectopia lentis, autosomal dominant Weill-Marchesani syndrome, MASS syndrome, and Shprintzen-Goldberg craniosynostosis syndrome. [provided by RefSeq, Jul 2008]

Function:
Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-1-containing microfibrils provide long-term force bearing structural support. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively.

Subcellular Location:
Secreted, extracellular space, extracellular matrix.

Post-translational modifications:
Forms intermolecular disulfide bonds either with other fibrillin-1 molecules or with other components of the microfibrils.