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由来生物
rabbit
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
形状
buffered aqueous glycerol solution
化学種の反応性
human
強化検証
independent
orthogonal RNAseq
Learn more about Antibody Enhanced Validation
テクニック
immunohistochemistry: 1:200- 1:500
免疫原配列
KTVIICKPTMSSAEIVAVDPDEPIHGPPFDFSLESSTSEVQRMWRLKAINDTAARLSYQNDPPFGSYVVPITVRDRLGMSSVTSLDVTLCDCITENDCTHRVDPRIGGGGVQLGKW
UniProtアクセッション番号
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 ... DSC2(1824)
詳細
Desmocollin 2 (DSC2) is a type I membrane glycoprotein that is localized in the cardiac tissue and other desmosome-containing tissues. It is expressed as two different splice variants with different carboxy-termini. DSC2a isoform possesses a larger cytoplasmic domain than the DSC2b isoform. The gene encoding this protein is present on chromosome 18. DSC2 contains four extracellular amino terminal domains, that is, an extracellular anchor domain (EA), a short transmembrane domain (TM), an intracellular anchor domain (IA), and an intracellular cadherin-binding domain (ICS). It has a single transmembrane domain and a cytoplasmic tail at the carboxy terminus.
免疫原
デスモコリン-2前駆体の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。
生物化学的／生理学的作用
Desmocollin 2 (DSC2) homo- and heterodimerize with each other and links the neighbouring cells by the interactions with the help of their extracellular cadherin domains. The domains also help these proteins to bind to desmogleins in a Ca2+-dependent manner. Its intracellular portions interact with plakoglobin (PG) and plakophilin-2 (PKP2). These two proteins in turn bind to desmoplakin (DSP) and provide a link to intermediate filaments. Mutations in the gene encoding DSC2 lead to arrhythmogenic right ventricular cardiomyopathy. DSC2 is essential for the establishment of early cardiac morphogenesis, normal myocardial structure and function and desmosome formation.
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 APREST72496
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
HPA012615-100UL:
HPA012615-25UL:
試験成績書（COA）
製品のロット番号・バッチ番号を入力して、試験成績書（COA） を検索できます。ロット番号・バッチ番号は、製品ラベルに「Lot」または「Batch」に続いて記載されています。
Arrhythmogenic right ventricular dysplasia/cardiomyopathy associated with mutations in the desmosomal gene desmocollin-2.
P Syriss
American Journal of Human Genetics, 79(5), 978-984 (2006)
Homozygous founder mutation in desmocollin-2 (DSC2) causes arrhythmogenic cardiomyopathy in the Hutterite population.
B Gerull et. al.
Circulation. Cardiovascular Genetics, 6(4), 327-336 (2013)
Brenda Gerull et al.
Circulation. Cardiovascular genetics, 6(4), 327-336 (2013-07-19)
Dominant mutations in cellular junction proteins are the major cause of arrhythmogenic cardiomyopathy, whereas recessive mutations in those proteins cause cardiocutaneous syndromes such as Naxos and Carvajal syndrome. The Hutterites are distinct genetic isolates who settled in North America in
Arnd Heuser et al.
American journal of human genetics, 79(6), 1081-1088 (2006-12-23)
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically heterogeneous heart-muscle disorder characterized by progressive fibrofatty replacement of right ventricular myocardium and an increased risk of sudden cardiac death. Mutations in desmosomal proteins that cause ARVC have been previously described; therefore
Katja Gehmlich et al.
Cardiovascular research, 90(1), 77-87 (2010-11-11)
Recent immunohistochemical studies observed the loss of plakoglobin (PG) from the intercalated disc (ID) as a hallmark of arrhythmogenic right ventricular cardiomyopathy (ARVC), suggesting a final common pathway for this disease. However, the underlying molecular processes are poorly understood. We
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