HPA006135
Anti-FLNC antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
別名:
Filamin C Antibody, Filamin C Antibody - Anti-FLNC antibody produced in rabbit, Anti-ABP-280-like protein, Anti-ABP-L, Anti-Actin-binding- like protein, Anti-Filamin-2, Anti-Filamin-C, Anti-Gamma-filamin, Anti-Protein FLNc
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About This Item
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由来生物
rabbit
品質水準
結合体
unconjugated
抗体製品の状態
affinity isolated antibody
抗体製品タイプ
primary antibodies
クローン
polyclonal
製品種目
Prestige Antibodies® Powered by Atlas Antibodies
フォーム
buffered aqueous glycerol solution
化学種の反応性
human
強化検証
orthogonal RNAseq
Learn more about Antibody Enhanced Validation
テクニック
immunofluorescence: 0.25-2 μg/mL
immunohistochemistry: 1:200-1:500
免疫原配列
HSLHETSTVLVETVTKSSSSRGSSYSSIPKFSSDASKVVTRGPGLSQAFVGQKNSFTVDCSKAGTNMMMVGVHGPKTPCEEVYVKHMGNRVYNVTYTVKEKGDY
UniProtアクセッション番号
アプリケーション
research pathology
輸送温度
wet ice
保管温度
−20°C
ターゲットの翻訳後修飾
unmodified
遺伝子情報
human ... FLNC(2318)
詳細
Filamin-C (FLN-C) or filamin-2 (FLN2) is a protein encoded by the FLNC gene in humans and is mapped to chromosome 7q32.1. It is referred to as ABPA, MFM5, MPD4. It belongs to the filamin family and is present in cardiac and skeletal muscle. Filamin-C comprises actin-binding domain (ABD) and 24 immunoglobulin-like repeats and is localized in the costameres, Z-disks, and intercalated disks of muscles
免疫原
フィラミン-CのPrEST(protein epitope signature tag)抗原リコンビナントタンパク質。
アプリケーション
Prestige抗体®は、Human Proteome Resource(HPR)プロジェクト(www.proteinatlas.org)によって開発・実証されたAtlas抗体です。抗体はすべて、数百の正常組織・疾病組織に対する免疫組織染色試験を行っています。これらの染色画像はHuman Protein Atlas(HPA)サイトで[Image Gallery]リンクをクリックするとご覧いただけます。さらに、ほとんどのPrestige抗体はプロテインアレイおよびウェスタンブロッティングの試験を行っています。試験のプロトコールおよびPrestige抗体、HPAに関する情報はsigma.com/prestigeをご覧ください。
All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information.
Anti-FLNC antibody produced in rabbit has been used in immunofluorescence and immunohistochemistry.
生物化学的／生理学的作用
Maintenance of the muscle structure filamin C plays an indispensable role in the maintenance of the structural integrity of cardiac and skeletal muscles for support against mechanical stress.
Mutation in this gene causes myofibrillar myopathy, characterized by myofibril disintegration and a huge formation of protein aggregates within skeletal muscle fibers.
特徴および利点
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
関連事項
Corresponding Antigen APREST70084
物理的形状
PBS溶液(pH 7.2, 40%グリセロールおよび0.02%アジ化ナトリウム含有)。
法的情報
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
免責事項
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.
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保管分類コード
10 - Combustible liquids
WGK
WGK 1
引火点(°F)
Not applicable
引火点(℃)
Not applicable
個人用保護具 (PPE)
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
適用法令
試験研究用途を考慮した関連法令を主に挙げております。化学物質以外については、一部の情報のみ提供しています。 製品を安全かつ合法的に使用することは、使用者の義務です。最新情報により修正される場合があります。WEBの反映には時間を要することがあるため、適宜SDSをご参照ください。
Jan Code
HPA006135-100UL:
HPA006135-25UL:
Matthias Vorgerd et al.
American journal of human genetics, 77(2), 297-304 (2005-06-02)
Myofibrillar myopathy (MFM) is a human disease that is characterized by focal myofibrillar destruction and pathological cytoplasmic protein aggregations. In an extended German pedigree with a novel form of MFM characterized by clinical features of a limb-girdle myopathy and morphological
Josefine de Stricker Borch et al.
Clinical neurology and neurosurgery, 176, 30-33 (2018-11-30)
We report three patients with a rare filamin C myofibrillar myopathy. They present with atypical symptoms that expand the phenotype of filaminopathy. The new findings are progressive contractures of muscles surrounding the temporomandibular joint, detailed single myofiber histology findings and
Rudolf A Kley et al.
Autophagy, 9(3), 422-423 (2012-12-15)
Myofibrillar myopathy caused by FLNC/filamin C mutations is characterized by disintegration of myofibrils and a massive formation of protein aggregates within skeletal muscle fibers. We performed immunofluorescence studies in skeletal muscle sections from filaminopathy patients to detect disturbances of protein
Thomas O Krag et al.
The Journal of clinical endocrinology and metabolism, 102(8), 2690-2700 (2017-04-30)
Glycogen storage disease (GSD) type XV is a rare disease caused by mutations in the GYG1 gene that codes for the core molecule of muscle glycogen, glycogenin 1. Nonetheless, glycogen is present in muscles of glycogenin 1-deficient patients, suggesting an
M Gariboldi et al.
Genomics, 21(2), 428-430 (1994-05-15)
Two genes encode actin-binding protein 280 isoforms. ABP-280 or filamin (FLN1) is present in the cytoskeleton of many cell types, whereas expression of FLN2 is limited to skeletal muscle and heart. FLN1 maps to human chromosome Xq28, and, by physical
ライフサイエンス、有機合成、材料科学、クロマトグラフィー、分析など、あらゆる分野の研究に経験のあるメンバーがおります。.
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