ARG62490

anti-Fibrillin 1 antibody [11C1.3]

anti-Fibrillin 1 antibody [11C1.3] for Electron microscopy,Flow cytometry,ICC/IF,IHC-Frozen sections,IHC-Formalin-fixed paraffin-embedded sections,Western blot and Human

Signaling Transduction antibody

概述

产品描述 Mouse Monoclonal antibody [11C1.3] recognizes Fibrillin 1
反应物种 Hu
应用 EM, FACS, ICC/IF, IHC-Fr, IHC-P, WB
宿主 Mouse
克隆 Monoclonal
克隆号 11C1.3
同位型 IgG1, kappa
靶点名称 Fibrillin 1
抗原物种 Bovine
抗原 Microfibrils from zonular apparatus of bovine eye.
抗原表位 Amino acids 451 - 909.
偶联标记 Un-conjugated
別名 ECTOL1; MFS1; WMS; SGS; SSKS; MASS; GPHYSD2; WMS2; ACMICD; OCTD; Fibrillin-1; FBN

应用说明

应用说明 WB: 1 - 2 µg/ml
FACS: 2µg for 106 cells
ICC/IF: 5 µg/ml
IHC: 1/10 - 1/500
* The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist.

属性

形式 Liquid
纯化 Protein G purified
缓冲液 1X PBS buffer with < 0.1% sodium azide.
抗菌剂 < 0.1% sodium azide.
浓度 2 mg/ml
存放说明 For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C or below. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use.
注意事项 For laboratory research only, not for drug, diagnostic or other use.

生物信息

数据库连接

GeneID: 2200 Human FBN1

Swiss-port # P35555 Human Fibrillin-1

基因名称 FBN1
全名 fibrillin 1
背景介绍 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]
生物功能 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 (By similarity). [UniProt]
细胞定位 Extracellular microfibrils
研究领域 Signaling Transduction antibody
预测分子量 312 kDa
翻译后修饰 Fibrillin-1: Cleavage of N- and C-terminus by furin is required for incorporation into the extracellular matrix and assembly into microfibrils (PubMed:27026396). The C-terminus, which corresponds to the Asprosin chain, was initially thought to constitute a propeptide (PubMed:24982166). Fibrillin-1 and Asprosin chains are still linked together during the secretion from cells, but are subsequently separated by furin, an essential step for incorporation of Fibrillin-1 into the nascent microfibrils (PubMed:24982166).
Fibrillin-1: Forms intermolecular disulfide bonds either with other fibrillin-1 molecules or with other components of the microfibrils.

克隆号文献

Epithelial-mesenchymal status influences how cells deposit fibrillin microfibrils.

ICC/IF / Human

Baldwin AK et al.
J Cell Sci.,  (2014)

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Latent transforming growth factor-β binding protein 2 negatively regulates coalescence of oxytalan fibers induced by stretching stress.

IHC / Human

Tsuruga E et al.
Connect Tissue Res.,  (2012)

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Discovery of putative oocyte quality markers by comparative ExacTag proteomics.

WB / Pig

Powell MD et al.
Proteomics Clin Appl.,  (2010)

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Dermal tissue and cellular expression of fibrillin-1 in diffuse cutaneous systemic sclerosis.

WB / Human

Wipff J et al.
Rheumatology (Oxford).,  (2010)

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Decreased gene expression of fibrillin-1 in stress urinary incontinence.

IHC / Human

Söderberg MW et al.
Neurourol Urodyn.,  (2010)

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Increased expression of extracellular matrix proteins in rapid atrial pacing-induced atrial fibrillation.

IHC / Pig

Lin CS et al.
Heart Rhythm.,  (2007)

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